Cystatin B: An intracellular cystatin subtype that is found in a broad variety of cell types. It is a cytosolic enzyme inhibitor that protects the cell against the proteolytic action of lysosomal enzymes such as CATHEPSINS.Cystatins: A homologous group of endogenous CYSTEINE PROTEINASE INHIBITORS. The cystatins inhibit most CYSTEINE ENDOPEPTIDASES such as PAPAIN, and other peptidases which have a sulfhydryl group at the active site.Cystatin C: An extracellular cystatin subtype that is abundantly expressed in bodily fluids. It may play a role in the inhibition of interstitial CYSTEINE PROTEASES.Unverricht-Lundborg Syndrome: An autosomal recessive condition characterized by recurrent myoclonic and generalized seizures, ATAXIA, slowly progressive intellectual deterioration, dysarthria, and intention tremor. Myoclonic seizures are severe and continuous, and tend to be triggered by movement, stress, and sensory stimuli. The age of onset is between 8 and 13 years, and the condition is relatively frequent in the Baltic region, especially Finland. (From Menkes, Textbook of Child Neurology, 5th ed, pp109-110)Myoclonic Epilepsies, Progressive: A heterogeneous group of primarily familial disorders characterized by myoclonic seizures, tonic-clonic seizures, ataxia, progressive intellectual deterioration, and neuronal degeneration. These include LAFORA DISEASE; MERRF SYNDROME; NEURONAL CEROID-LIPOFUSCINOSIS; sialidosis (see MUCOLIPIDOSES), and UNVERRICHT-LUNDBORG SYNDROME.Cystatin A: A cytastin subtype found at high levels in the SKIN and in BLOOD CELLS. Cystatin A incorporates into the cornified cell envelope of stratified squamous epithelial cells and may play a role in bacteriostatic properties of skin.Epilepsies, Myoclonic: A clinically diverse group of epilepsy syndromes characterized either by myoclonic seizures or by myoclonus in association with other seizure types. Myoclonic epilepsy syndromes are divided into three subtypes based on etiology: familial, cryptogenic, and symptomatic (i.e., occurring secondary to known disease processes such as infections, hypoxic-ischemic injuries, trauma, etc.).Cathepsin B: A lysosomal cysteine proteinase with a specificity similar to that of PAPAIN. The enzyme is present in a variety of tissues and is important in many physiological and pathological processes. In pathology, cathepsin B has been found to be involved in DEMYELINATION; EMPHYSEMA; RHEUMATOID ARTHRITIS, and NEOPLASM INVASIVENESS.Cysteine Proteinase Inhibitors: Exogenous and endogenous compounds which inhibit CYSTEINE ENDOPEPTIDASES.Micrococcus luteus: A species of gram-positive, spherical bacteria whose organisms occur in tetrads and in irregular clusters of tetrads. The primary habitat is mammalian skin.Reducing Agents: Materials that add an electron to an element or compound, that is, decrease the positiveness of its valence. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 5th ed)Papain: A proteolytic enzyme obtained from Carica papaya. It is also the name used for a purified mixture of papain and CHYMOPAPAIN that is used as a topical enzymatic debriding agent. EC 3.4.22.2.Galactosides: Glycosides formed by the reaction of the hydroxyl group on the anomeric carbon atom of galactose with an alcohol to form an acetal. They include both alpha- and beta-galactosides.Cystathionine gamma-Lyase: A multifunctional pyridoxal phosphate enzyme. In the final step in the biosynthesis of cysteine it catalyzes the cleavage of cystathionine to yield cysteine, ammonia, and 2-ketobutyrate. EC 4.4.1.1.Luciferases, Firefly: Luciferases from FIREFLIES, usually Photinus, that oxidizes FIREFLY LUCIFERIN to cause emission of PHOTONS.Salivary Cystatins: A group of closely-related cystatins found in SALIVA.Cathepsins: A group of lysosomal proteinases or endopeptidases found in aqueous extracts of a variety of animal tissues. They function optimally within an acidic pH range. The cathepsins occur as a variety of enzyme subtypes including SERINE PROTEASES; ASPARTIC PROTEINASES; and CYSTEINE PROTEASES.Vault Ribonucleoprotein Particles: Large cytoplasmic ribonucleoprotein particles that have an eight-fold symmetry with a central pore and petal-like structure giving the appearance of an octagonal dome. (The Dictionary of Cell Biology, Lackie and Dow, 2nd ed.)

Altered spacing of promoter elements due to the dodecamer repeat expansion contributes to reduced expression of the cystatin B gene in EPM1. (1/74)

Progressive myoclonus epilepsy of the Unverricht-Lundborg type (EPM1; MIM 254800) is an autosomal recessive disorder characterized by seizures, myoclonus and progression to cerebellar ataxia. EPM1 arises due to mutations in the cystatin B (CSTB) gene which encodes a cysteine proteinase inhibitor. Only a minority of EPM1 alleles carry point mutations, while the majority contain large expansions of the dodecamer CCCCGCCCCGCG repeat which is present at two to three copies in normal individuals. The dodecamer repeat is located in the 5' flanking region of the CSTB gene, presumably in its promoter. The pathological repeat expansion results in a reduction in CSTB mRNA, which may be cell specific. To elucidate the mechanism of this reduction of gene expression, we have studied the putative CSTB promoter in vitro. A 3.8 kb fragment, containing the putative promoter with a 600 bp repeat expansion, showed a 2- to 4-fold reduction in luciferase activity compared with an identical fragment with a normal repeat; this reduction was observed only in certain cell types. Introduction of heterologous DNA fragments of 730 and 1000 bp into the normal promoter, instead of the repeat expansion, showed similarly reduced activity. Terminal deletions of the promoter implicate a putative AP-1 binding site, upstream of the repeat, in CSTB transcription activation. We propose that a novel mechanism of pathogenesis, the altering of the spacing of transcription factor binding sites from each other and/or the transcription initiation site due to repeat expansion, is among the causes of reduction in CSTB expression and thus EPM1.  (+info)

Instability of the EPM1 minisatellite. (2/74)

Inherited mutations in the cystatin B gene ( CSTB ) are responsible for progressive myoclonus epilepsy type 1 (EPM1; MIM 254800). This autosomal recessive disease is characterized by variable progression to mental retardation, dementia and ataxia. The majority of EPM1 alleles identified to date contain expansions of a dodecamer repeat located upstream of the transcription start site of the CSTB gene. Normal alleles contain two or three copies of the repeat, whereas pathogenic alleles contain >40 repeats. We examined the meiotic stability of pathogenic, expanded EPM1 alleles from 17 EPM1 families by employing a fluorescence-based PCR-based genotyping assay capable of detecting single dodecamer repeat unit differences on an automated DNA sequencer. We followed 74 expanded allele transmissions to 30 affected individuals and 22 carriers. Thirty-five of 74 expanded allele transmissions demonstrated either contraction or expansion of the minisatellite, typically by a single repeat unit. Thus expanded alleles of the EPM1 minisatellite demonstrate a mutation rate of 47%, the highest yet observed for pathogenetic alleles of a human minisatellite.  (+info)

Cysteine proteinase inhibitors stefin A, stefin B, and cystatin C in sera from patients with colorectal cancer: relation to prognosis. (3/74)

The levels of cysteine proteinase inhibitors stefin A, stefin B, and cystatin C were determined using ELISAs in sera obtained preoperatively from 345 patients with colorectal cancer and in control sera from 125 healthy blood donors. The levels of stefin A and cystatin C were found to be moderately increased in patient sera (1.4-fold and 1.6-fold, respectively; P < 0.0001), whereas the level of stefin B remained statistically unchanged when compared with controls. The medians were 4.3 ng/ml versus 3.2 ng/ml for stefin A, 1.2 ng/ml versus 1.7 ng/ml for stefin B, and 679 ng/ml versus 425 ng/ml for cystatin C. In patient sera, a weak correlation of cystatin C with age (r = 0.34; P < 0.001) and gender (P = 0.01) was found. Stefin A and cystatin C levels were independent of Dukes' stage, whereas stefin B correlated significantly with Dukes' stage, its level being the highest in stage D (P < 0.007). Stefin B and cystatin C correlated with survival, whereas stefin A was not a significant prognostic factor in this study. Using medians as cutoff values, patients with high levels of stefin B and patients with high levels of cystatin C exhibited a significantly higher risk of death than those with low levels of inhibitors (hazard ratio = 1.6; 95% confidence interval, 1.2-2.2; P = 0.002 for stefin B; hazard ratio = 1.3; 95% confidence interval, 1.0-1.8; P = 0.04 for cystatin C). Our results reveal a correlation between high levels of extracellular cysteine proteinase inhibitors and short survival in patients with colorectal cancer, and the data thus support previous studies suggesting a contributing role of protease inhibitors in the progression of cancer.  (+info)

Structural basis of the endoproteinase-protein inhibitor interaction. (4/74)

Proteolytic enzymes are potentially hazardous to their protein environment, so that their activity must be carefully controlled. Living organisms use protein inhibitors as a major tool to regulate the proteolytic activity of proteinases. Most of the inhibitors for which 3D structures are available are directed towards serine proteinases, interacting with the active sites in a 'canonical' i.e. substrate-like manner via an exposed reactive site loop of conserved conformation. More recently, some non-canonically binding serine proteinase inhibitors directed against coagulation factors, in particular thrombin, a few cysteine proteinase inhibitors inhibitory towards papain-like proteinases, and three zinc endopeptidase inhibitors directed against metzincins and thermolysin have been characterised in the free and complexed state, displaying novel mechanisms of inhibition with their target proteinases. These different interaction modes are presented and briefly discussed with respect to the different strategies applied by nature.  (+info)

Prognostic significance of cysteine proteinases cathepsins B and L and their endogenous inhibitors stefins A and B in patients with squamous cell carcinoma of the head and neck. (5/74)

Cysteine proteinases cathepsins (Cats) B and L and their endogenous inhibitors stefins (Stefs) A and B are implicated in the processes of local and metastatic tumor spread. They were identified as potential prognosticators in various malignant diseases, particularly in breast cancer. The aim of the present study was to determine the concentrations of Cats B and L and Stefs A and B in the tumor and adjacent normal tissue samples collected from 49 patients (the present group) with squamous cell carcinoma of the head and neck (SCCHN), using quantitative immunosorbent assays (ELISA; KRKA d.d., Novo mesto, Slovenia). Their clinical significance was compared with that from a previous study (the reference group, 45 patients; Budihna et al., Biol. Chem. Hoppe-Seyler, 377: 385-390, 1996). The follow-up of patients from the latter report was updated for this purpose. In the present group, significantly higher concentrations of Cat B (P < 0.0001), Cat L (P < 0.0001) and Stef A (P = 0.006) were found in tumors compared with concentrations in their normal tissue counterparts. Cat concentrations in normal laryngeal tissue were significantly/marginally elevated compared with nonlaryngeal tissue (Cat B, P = 0.02; Cat L, P = 0.06). The tumor concentration of Cat L was found to correlate with pT classification (P = 0.005) and tumor-node-metastasis stage (P = 0.05), whereas the concentrations of Stefs A and B correlated with pN classification (P = 0.007 and P = 0.03, respectively) and tumor-node-metastasis stage of the disease (P = 0.02 and P = 0.03, respectively). There was no statistically significant difference between low and high Cat B or Cat L groups, regarding either disease-free survival or disease-specific survival, using a minimum P approach to determine cutoff concentrations. The risk of disease recurrence and SCCHN-related death was significantly higher in patients with low Stef A (P = 0.0006 and P = 0.0005, respectively) and Stef B (P = 0.0009 and P = 0.0007, respectively) tumors, compared with those with high-Stef A and Stef B tumors. These results remained significant even after Ps were adjusted for a possible bias in the estimated effect on survival. The survival analysis in the reference group also confirmed these findings (Stef A: P = 0.0009 and P = 0.002, respectively; Stef B: P = 0.03 and P = 0.009, respectively). To avoid any possible bias arising from the differences between the laboratories that performed the biochemical analysis, the concentrations of both Stefs in the present group and in the reference group were standardized and coupled together to form a uniform group. In univariate survival analysis, standardized values of Stef A and Stef B correlated inversely with the rate of relapse (P = 0.0000) and mortality rate (P = 0.0000). Multivariate regression analysis showed that the standardized value of Stef A is the strongest independent prognostic factor for both disease-free survival and disease-specific survival. These findings show the specific role of Cats B and L and Stefs A and B in the invasive behavior of SCCHN. Furthermore, Stef A proved to be a reliable prognosticator of the risk of relapse and death in patients with this type of cancer.  (+info)

Gene expression profiles in normal and Otx2-/- early gastrulating mouse embryos. (6/74)

The mouse Otx2 gene is a homeobox transcription factor required as early as gastrulation for the proper development of the head. We compared gene expression profiles in wild-type and Otx2(-/-) 6.5 days postcoitum embryos by using a serial analysis of gene expression assay adapted to microdissected structures. Among a broader list, the study of six genes found to be differentially expressed allows defining a role for Otx2 in the orchestration of cell movements leading to the adequate organization of the embryo before gastrulation.  (+info)

Role of the single cysteine residue, Cys 3, of human and bovine cystatin B (stefin B) in the inhibition of cysteine proteinases. (7/74)

Cystatin B is unique among cysteine proteinase inhibitors of the cystatin superfamily in having a free Cys in the N-terminal segment of the proteinase binding region. The importance of this residue for inhibition of target proteinases was assessed by studies of the affinity and kinetics of interaction of human and bovine wild-type cystatin B and the Cys 3-to-Ser mutants of the inhibitors with papain and cathepsins L, H, and B. The wild-type forms from the two species had about the same affinity for each proteinase, binding tightly to papain and cathepsin L and more weakly to cathepsins H and B. In general, these affinities were appreciably higher than those reported earlier, perhaps because of irreversible oxidation of Cys 3 in previous work. The Cys-to-Ser mutation resulted in weaker binding of cystatin B to all four proteinases examined, the effect varying with both the proteinase and the species variant of the inhibitor. The affinities of the human inhibitor for papain and cathepsin H were decreased by threefold to fourfold and that for cathepsin B by approximately 20-fold, whereas the reductions in the affinities of the bovine inhibitor for papain and cathepsins H and B were approximately 14-fold, approximately 10-fold and approximately 300-fold, respectively. The decreases in affinity for cathepsin L could not be properly quantified but were greater than threefold. Increased dissociation rate constants were responsible for the weaker binding of both mutants to papain. By contrast, the reduced affinities for cathepsins H and B were due to decreased association rate constants. Cys 3 of both human and bovine cystatin B is thus of appreciable importance for inhibition of cysteine proteinases, in particular cathepsin B.  (+info)

Cystatin B-deficient mice have increased expression of apoptosis and glial activation genes. (8/74)

Loss-of-function mutations in the cystatin B (Cstb) gene cause a neurological disorder known as Unverricht-Lundborg disease (EPM1) in human patients. Mice that lack Cstb provide a mammalian model for EPM1 by displaying progressive ataxia and myoclonic seizures. We analyzed RNAs from brains of Cstb-deficient mice by using modified differential display, oligonucleotide microarray hybridization and quantitative reverse transcriptase polymerase chain reaction to examine the molecular consequences of the lack of Cstb. We identified seven genes that have consistently increased transcript levels in neurological tissues from the knockout mice. These genes are cathepsin S, C1q B-chain of complement (C1qB), beta2-microglobulin, glial fibrillary acidic protein (Gfap), apolipoprotein D, fibronectin 1 and metallothionein II, which are expected to be involved in increased proteolysis, apoptosis and glial activation. The molecular changes in Cstb-deficient mice are consistent with the pathology found in the mouse model and may provide clues towards the identification of therapeutic points of intervention for EPM1 patients.  (+info)

*Unverricht-Lundborg disease

Cystatin B Characteristics[edit]. A recent study has attempted to describe the behavior of normal and mutated cystatin B as it ... The results show that cystatin B has a polymeric structure, and that the mutated form of cystatin B, which is present in ... The cause of ULD is known to be a mutation of the gene that produces cystatin B.[2] The disease is autosomal recessive, so both ... Current research links cystatin B to production of inhibitory neurons known as GABAergic neurons. It has shown that a lack of ...

*Chromosome 20

CST9L: Cystatin-9-like. *CSTL1: Cystatin-like 1. *CTCFL: CCCTC-binding factor-like ...

*Egg white

Cystatin. 0.05% Ovalbumin is the most abundant protein in albumen. Classed as phosphoglycoprotein, during storage, it converts ...

*Cathepsin D

Lenarcic B, Krasovec M, Ritonja A, Olafsson I, Turk V (March 1991). "Inactivation of human cystatin C and kininogen by human ...

*Creatinine

Cystatin C - novel marker of kidney function. *Jaffe reaction - an example of creatinine assay methodology ...

*Evolution of snake venom

A]: crotamine and cystatin. [B]: 12 toxin families, including CRiSPs and nerve growth factors. [C]: 2 toxin families, including ...

*Peptide transporter carbon starvation family

Cystatin A Schultz, JE; Matin, A (March 5, 1991). "Molecular and functional characterization of a carbon starvation gene of ...

*Kininogen

Closely related proteins include cystatin. Stefan Offermanns; Walter Rosenthal (2008). Encyclopedia of Molecular Pharmacology. ...

*CST7 (gene)

1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-F is a protein that in humans is encoded by the CST7 gene. The cystatin superfamily encompasses proteins that contain ... The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions. ... 2004). "Cystatin F is secreted, but artificial modification of its C-terminus can induce its endocytic targeting". Exp. Cell ...

*CSTL1

1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-like 1 is a protein that in humans is encoded by the CSTL1 gene. The cystatin superfamily encompasses proteins that ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located at ... The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions. ...

*Renal function

Cystatin C is freely filtered at the glomerulus. After filtration, Cystatin C is reabsorbed and catabolized by the tubular ... Cystatin C levels are therefore measured not in the urine, but in the bloodstream. Equations have been developed linking ... One of these is cystatin C, a ubiquitous protein secreted by most cells in the body (it is an inhibitor of cysteine protease). ... The most accurate is (sex, age and race) adjusted cystatin C, followed by (sex, age and race) adjusted creatinine and then ...

*Fetuin

... s are members of a family of proteins that evolved from the protein cystatin by gene duplication and exchange of gene ... Fetuins thus belong to the cystatin superfamily of proteins. Fetuin relatives within this superfamily are the histidine-rich ...

*CST6 (gene)

1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-M is a protein that in humans is encoded by the CST6 gene. The cystatin superfamily encompasses proteins that contain ... 2006). "Cystatin M/E is a high affinity inhibitor of cathepsin V and cathepsin L by a reactive site that is distinct from the ... The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions, ...

*Rhipicephalus haemaphysaloides

"Functional characterization of a cystatin from the tick Rhipicephalus haemaphysaloides". Parasites & Vectors. Retrieved 11 ...

*Genetically modified tomato

Chan, Y.; Yang, A.; Chen, J.; Yeh, K.; Chan, M. (2010). "Heterologous expression of taro cystatin protects transgenic tomato ...

*CST4

... cystatin SN, cystatin SA, cystatin S, and two phosphorylated forms of cystatin S) in human whole saliva and determination of ... 1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-S is a protein that in humans is encoded by the CST4 gene. The cystatin superfamily encompasses proteins that contain ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in ...

*CST5

1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-D is a protein that in humans is encoded by the CST5 gene. The cystatin superfamily encompasses proteins that contain ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in ... The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions. ...

*Cotesia congregata

The fourth group are cystatin proteins, which will inhibit cysteine proteases. These will inhibit the breakdown of the proteins ...

*CST1

... cystatin SN, cystatin SA, cystatin S, and two phosphorylated forms of cystatin S) in human whole saliva and determination of ... 1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-SN is a protein that in humans is encoded by the CST1 gene. The cystatin superfamily encompasses proteins that contain ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in ...

*CST8 (gene)

1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-8 is a protein that in humans is encoded by the CST8 gene. The cystatin superfamily encompasses proteins that contain ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in ... "Entrez Gene: CST8 cystatin 8 (cystatin-related epididymal specific)". The MEROPS online database for peptidases and their ...

*CST2

... cystatin SN, cystatin SA, cystatin S, and two phosphorylated forms of cystatin S) in human whole saliva and determination of ... 1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-SA is a protein that in humans is encoded by the CST2 gene. The cystatin superfamily encompasses proteins that contain ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in ...

*CST9L

1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... Cystatin-9-like is a protein that in humans is encoded by the CST9L gene. The cystatin superfamily encompasses proteins that ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in ... The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions. ...

*CST11

1989). "Cystatin superfamily. Evidence that family II cystatin genes are evolutionarily related to family III cystatin genes". ... The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in ... Cystatin-11 is a protein that in humans is encoded by the CST11 gene. The cystatin superfamily encompasses proteins that ... 2002). "Cystatin 11: a new member of the cystatin type 2 family". Endocrinology. 143 (7): 2787-96. doi:10.1210/en.143.7.2787. ...

*Staphopain A (Staphylococcus aureus)

Staphopain A is inhibited by phosphorylated cystatin α and α2-macroglobulin. Staphopain A can cleave and lower the activity α1- ... Inhibition of staphopain A by phosphorylated cystatin α did prevent colony formation in skin tissue, but the effect could also ... "Inhibition of cysteine protease and growth of Staphylococcus aureus V8 and poliovirus by phosphorylated cystatin alpha ... properties of peptidyl derivatives and cyclopeptides structurally based upon the inhibitory centre of human cystatin C. ...

*Clinical Biochemistry

Juliana F. Roos; Jenny Doust; Susan E. Tett; Carl M.J. Kirkpatrick (2007). "Diagnostic accuracy of cystatin C compared to serum ... serum cystatin C or equations?". Clinical Biochemistry. 40 (3-4): 153-161. doi:10.1016/j.clinbiochem.2006.10.014. ...

*Reference ranges for blood tests

Finney H, Newman DJ, Price CP (January 2000). "Adult reference ranges for serum cystatin C, creatinine and predicted creatinine ...
Cystatin B antibody for detecting human Cystatin B. Validated on up to 12 cell lysates for western blotting. Try a trial size today.
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Cystatin B: An intracellular cystatin subtype that is found in a broad variety of cell types. It is a cytosolic enzyme inhibitor that protects the cell against the proteolytic action of lysosomal enzymes such as CATHEPSINS.
SpecificityC TerminusStorage/StabilityAliquot and store at -20°C Minimize freezing and thawing More InformationImmunogenThe immunogen was a 13-residue peptide matching a sequence from the C Terminus of Human CSTB See Accession Number s NP_000091 1 Formulation 0 5 mg/ml in TBS 0 02% NaNH3 pH7
The following experiment in e-mail publishing contains: Spirov, A.V. (1996). Self-assemblage of gene nets in evolution via recruiting of new netters. Bull. Can. Soc. Theor. Biol. plus a commentary I wrote. Thought some of you might be interested in continuing this dialogue. -Dick Gordon ---------- Forwarded message ---------- Date: Fri, 19 Jul 1996 12:29:52 +0600 From: Denis Thieffry ,denis at cifn.unam.mx, To: cstb at scotia.dfo.ca Subject: CSTB Bulletin - Spring 96 Issue Dear colleague, The new issue of the CSTB Bulletin is now available on the web. You will find a link to it in the CSTB Home page, at the url: http://biome.bio.ns.ca/science/cstb/cstb.html We are including below the contents of this 96Spring issue, along the editorial and the abstract of a long and rich paper by Alexander SPIROV. Best regards, Denis Thieffry (CSTB Bulletin Editor) ***************************************************** CSTB BULLETIN SPRING 96 - CONTENTS ***************************************************** ...
Plotted over groups are mean ± SEM of EPM and hole board behaviors. Means markedwith a different letter differ from each other (P | .05). (a), shows freque
Mutations in Lafora disease genes (laforin and malin) and in Univerricht-Lundborg PME genes (cystatin B) are inherited in an autosomal recessive fashion. The gene EPM2A makes an enzyme called dual specificity protein phosphatase or laforin, and the gene EPM2B makes another enzyme named ubiquitin E3 ligase or malin. Therefore, most cases of Lafora PMEs are enzymopathies, are classified as lysosomal diseases, and produce a system-wide glycogen storage disorder. A minority of cases of Lafora disease are caused by as yet unidentified genes. Cystatin B is a cysteine protease inhibitor and its deficiency causes abnormal activation of cathepsin S. G1qB-chain of complement, beta2-microglubulin, glial fibrillary acidic protein, apolipoprotein D, fibronectin 1, and metallothionein II, which are factors involved in proteolysis, apoptosis, and glial activation (Leiuallen et al., 2001). The lysosome-associated functions of cystatin B are also associated with the pathogenic mutations, suggesting that ...
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Alignment of cystatin superfamily domain.The domain was aligned using ClustalW and adjusted manually. (A) The three parts of the papain binding domain (G, QXVXG
Germline/De novo/Somatic: Genetic origin of variant: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc ...
Germline/De novo/Somatic: Genetic origin of variant: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc ...
Ravi on olemas haigus, aga kui loomaarst kahtlustab EPM, ta võib panna oma hobuse antibiootikum raviskeemi, teadmata, kas hobune on antikehad tema seljaajuvedelik.Enamik hobuseid parandab nelja nädala jooksul, kuid kui seal on mingit vastust kahe nädala jooksul ravi alustamisest, hobune peaks läbima seljaajuvedelikust test täiuslikum diagnoosi.Kui hobune ei reageeri antibiootikumidele, ootame ravi kestab 90 päeva või rohkem.Loomaarst võib ka hallata põletikuvastaste vähendada turse kudedes hobuse kesknärvisüsteemi.Ta võib soovitada, E-vitamiini või muid toidulisandeid.. Kahjuks ainult umbes 25 protsenti hobused taastuda täielikult EPM, muutudes naasta nende varasemast tööst. hobustele EPM sageli ägenemist , ja see on eriti tõsi, kui nad ei ole saanud täiskomplekti antibiootikumravi.. ...
This is a guide to EPM in horses. You can read of its prevention, whether it is contagious, which is the prognosis, if its curable and much more.
Progressive myoclonus eplilepsy or Unverricht-Lundborg disease is a recessively inherited epileptic disorder of young children and adults due to misfunction of cystatin B. Detailed pathogenetic mechanisms are still unknown. First clinical symptoms occur after a normal infancy and early childhood at the age of 6 to 15 years presenting with epileptic seizures followed soon later by myoclonic jerks. Attacks are easily provoked by varied stimuli, particularly after waking in the morning. The course of the disease is progressive and without proper treatment slowly leads to decline of mental and motor functions. Spike-and-wave pattern is typical finding of EEG recording.
Cystatin-SN is a protein that in humans is encoded by the CST1 gene. The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and the kininogens. The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions, where they appear to provide protective functions. The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in the cystatin locus and encodes a cysteine proteinase inhibitor found in saliva, tears, urine, and seminal fluid. GRCh38: Ensembl release 89: ENSG00000170373 - Ensembl, May 2017 "Human PubMed Reference:". "Entrez Gene: CST1 cystatin SN". The MEROPS online database for ...
Bombyx cysteine proteinase inhibitor: Bombyx cysteine proteinase inhibitor (BCPI) from the hemolymph of Bombyx mori; alpha and beta are two forms differing only in three amino acid residues at N terminal; amino acid sequence in first source
The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and the kininogens. The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions. The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in the cystatin locus and encodes a type 2 salivary cysteine peptidase inhibitor. The protein is an S-type cystatin, based on its high level of expression in saliva, tears and seminal plasma. The specific role in these fluids is unclear but antibacterial and antiviral activity is present, consistent with a protective function. [provided by RefSeq, Jul 2008 ...
1N9J: Three-dimensional domain swapping in the folded and molten-globule states of cystatins, an amyloid-forming structural superfamily
Horse owners with EPM horses often have questions about alternative treatments, new diagnostic tests and the status of research into EPM. We asked Dr. David
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Human APP PicoKine™ ELISA Kit (Amyloid beta A4 protein)...: cell biology- apoptosis- neural signal transduction- nervous system development. Bazı Hormon Belirteçleri için Yeni Çıkan ELISA Kitleri: -Alpha (aCGRP) Calcitonin Gene-Related Peptide ELISA Kit. -Beta(bCGRP) Calcitonin Gene-Related Peptide ELISA Kit. -Bradykinin ELISA Kit. -Caerulein ELISA Kit. -Dynorphin ELISA Kit. -ELABELA ELISA kit. -Enkephalin ELISA Kit. -Exendin-4 ELISA Kit. -Galanin ELISA Kit. -Hepsidin 25 ELISA Kit. -LHRH ELISA Kit. -Liraglutide ELISA Kit. -Pancreastatin ELISA Kit. -Secretin ELISA Kit. -TIP39(Tuberoinfundibular peptide39) ELISA Kit. -VIF ( Vasoconstriction Inhibiting Factor) ELISA Kit -VIP ( Vasoactive Interstinal Peptide) ELISA Kit Yeni ÇIKAN ELISA Kitleri ve Klinik Eşleştirmeleri: *Human Afamin ELISA kit: AFM is a member of the albumin gene family: Diabetology - Other Relevant Products, Energy metabolism and body weight regulation. *Human Cystatin B ELISA: Autoimmunity, Oncolog. *Human Cysteine-Rich ...
The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG repeat from 2-3 copies to 30-78 copies. [provided by RefSeq, Jul 2016 ...
The cystatins are a family of cysteine protease inhibitors which share a sequence homology and a common tertiary structure of an alpha helix lying on top of an anti-parallel beta sheet. The family is subdivided as described below. Cystatins show similarity to fetuins, kininogens, histidine-rich glycoproteins and cystatin-related proteins. Cystatins mainly inhibit peptidase enzymes (another term for proteases) belonging to peptidase families C1 (papain family) and C13 (legumain family). They are known to mis-fold to form amyloid deposits and are implicated in several diseases.[citation needed] The cystatin family includes: The Type 1 cystatins, which are intracellular and are present in the cytosol of many cell types, but can also appear in body fluids at significant concentrations. They are single-chain polypeptides of about 100 residues, which have neither disulfide bonds nor carbohydrate side-chains. Type 1 cystatins are also known as Stefins (after the Stefan Institute where they were first ...
The precursor protein in cystatin C amyloidosis (ACys) is cystatin C, which is a cysteine protease inhibitor that contains a point mutation. This condition is clinically termed HCHWA, Icelandic type. ... more
Cystatin C protein (recombinant) can be used as antigen in immuno-based applications. It can also be used to formulate calibrators and controls. Size: 1 mg.
4) Institute of Chemistry, Slovak Academy of Sciences, Bratislava, Slovakia.. Address for correspondence: O.N. Poteryaeva, Russian Academy of Medical Sciences, Institute of Physiology, Novosibirsk, Russia.. Summary: Cystatin C is the best known extracellular endogenous cysteine proteinase inhibitor and has been studied as a possible index of tumor growth and as a marker of the effectiveness of antitumor therapy. The aim of this study was to evaluate cystatin C concentrations in murine tumor tissues (compared with other organs not directly involved with tumor development, such as the liver and spleen) during treatment with several antitumor drugs (Ukrain and/or cyclophosphane). Cystatin C concentrations in murine tissues and biological fluids was determined by enzyme-linked immunosorbent (ELISA) assay The cystatin C ELISA test is a sandwich immunoassay, which uses immobilized rabbit antihuman cystatin C Pab and mouse antihuman cystatin C Mab-HRP (monoclonal antibodies, conjugated with horseradish ...
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Background: Action myoclonus-renal failure syndrome is a hereditary form of progressive myoclonus epilepsy associated with renal failure. It is considered to be an autosomal-recessive disease related to loss-of-function mutations in SCARB2. We studied a German AMRF family, additionally showing signs of demyelinating polyneuropathy and dilated cardiomyopathy. To test the hypothesis whether isolated appearance of individual AMRF syndrome features could be related to heterozygote SCARB2 mutations, we screened for SCARB2 mutations in unrelated patients showing isolated AMRF features. Methods: In the AMRF family all exons of SCARB2 were analyzed by Sanger sequencing. The mutation screening of unrelated patients with isolated AMRF features affected by either epilepsy (n = 103, progressive myoclonus epilepsy or generalized epilepsy), demyelinating polyneuropathy (n = 103), renal failure (n = 192) or dilated cardiomyopathy (n = 85) was performed as high resolution melting curve analysis of the SCARB2 ...
EPM1 (epilepsy, progressive myoclonic 1; Unverricht-Lundborg disease, OMIM #254800) is the most frequent form of progressive myoclonus epilepsy. Previous findings have suggested that its...
The median cystatin C value was 0.83 mg/L. Increasing quartiles of cystatin C were strongly associated with death and MI (6.9%, 7.1%, 9.5%, and 16.2%). The fully adjusted hazard ratios per standard deviation of cystatin C in the non-ST-segment elevation (NSTE)-ACS and STE-ACS populations were 1.12 (95% confidence interval [CI], 1.04-1.20) (n = 8,053) and 1.06 (95% CI, 0.97-1.17) (n = 5,278), respectively. However, the overall C-statistics for the different renal function measurements were minimally different, ranging from 0.6923 (cystatin C) to 0.6941 (CKD-EPI). Neither cystatin C nor other creatinine-based equations increased the area under the curve compared to a model that did not include a renal marker. The benefit of ticagrelor tended to be greater with higher levels of cystatin C.. ...
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Treatment of myoclonus focuses on medications that may help reduce symptoms. Physiological myoclonus does not require specific treatment.
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Laforas disease (LD; OMIM 254780) is an autosomal recessive form of progressive myoclonus epilepsy characterized by seizures and cumulative neurological deterioration. Onset occurs during late childhood and usually results in death within ten years of the first symptoms1,2. With few exceptions, patients follow a homogeneous clinical course despite the existence of genetic heterogeneity3. Biopsy of various tissues, including brain, revealed characteristic polyglucosan inclusions called Lafora bodies4-8, which suggested LD might be a generalized storage disease6,9. Using a positional cloning approach, we have identified at chromosome 6q24 a novel gene, EPM2A, that encodes a protein with consensus amino acid sequence indicative of a protein tyrosine phosphatase (PTP). mRNA transcripts representing alternatively spliced forms of EPM2A were found in every tissue examined, including brain. Six distinct DNA sequence variations in EPM2A in nine families, and one homozygous microdeletion in another ...
Background. High activity of cysteine proteases such as legumain and the cathepsins have been shown to facilitate growth and invasion of a variety of tumor types. In breast cancer, several recent studies have indicated that loss of the cysteine protease inhibitor cystatin E/M leads to increased growth and metastasis. Although cystatin E/M is normally expressed in the skin, its role in cysteine protease regulation and progression of malignant melanoma has not been studied.. Methods. A panel of various non-melanoma and melanoma cell lines was used. Cystatin E/M and C were analyzed in cell media by immunoblotting and ELISA. Legumain, cathepsin B and L were analyzed in cell lysates by immunoblotting and their enzymatic activities were analyzed by peptide substrates. Two melanoma cell lines lacking detectable secretion of cystatin E/M were transfected with a cystatin E/M expression plasmid (pCST6), and migration and invasiveness were studied by a Matrigel invasion assay.. Results. Cystatin E/M was ...
Muona, Mikko; Berkovic, Samuel F.; Dibbens, Leanne M.; Oliver, Karen L.; Maljevic, Snezana; Bayly, Marta A.; Joensuu, Tarja; Canafoglia, Laura; Franceschetti, Silvana; Michelucci, Roberto; Markkinen, Salla; Heron, Sarah E.; Hildebrand, Michael S.; Andermann, Eva; Andermann, Frederick; Gambardella, Antonio; Tinuper, Paolo; Licchetta, Laura; Scheffer, Ingrid E.; Criscuolo, Chiara; Filla, Alessandro; Ferlazzo, Edoardo; Ahmad, Jamil; Ahmad, Adeel; Baykan, Betul; Said, Edith; Topcu, Meral; Riguzzi, Patrizia; King, Mary D.; Ozkara, Cigdem; Andrade, Danielle M.; Engelsen, Bernt; Crespel, Arielle; Lindenau, Matthias; Lohmann, Ebba; Saletti, Veronica; Massano, João; Privitera, Michael; Espay, Alberto J.; Kauffmann, Birgit; Duchowny, Michael; Møller, Rikke S.; Straussberg, Rachel; Afawi, Zaid; Ben-Zeev, Bruria; Samocha, Kaitlin E.; Daly, Mark J.; Petrou, Steven; Lerche, Holger; Palotie, Aarno; Lehesjoki, Anna-Elina. 2015. A recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy. ...
Absence of the protease inhibitor cystatin C in inflammatory cells results in larger plaque area in plaque regression of apoE-deficient mice ...
SNARE proteins participate in recognition and fusion of membranes. A SNARE complex consisting of vti1b, syntaxin 8, syntaxin 7, and endobrevin/VAMP-8 which is required for fusion of late endosomes in vitro has been identified recently. Here, we generated mice deficient in vti1b to study the function of this protein in vivo. vti1b-deficient mice had reduced amounts of syntaxin 8 due to degradation of the syntaxin 8 protein, while the amounts of syntaxin 7 and endobrevin did not change. These data indicate that vti1b is specifically required for the stability of a single SNARE partner. vti1b-deficient mice were viable and fertile. Most vti1b-deficient mice were indistinguishable from wild-type mice and did not display defects in transport to the lysosome. However, 20% of the vti1b-deficient mice were smaller. Lysosomal degradation of an endocytosed protein was slightly delayed in hepatocytes derived from these mice. Multivesicular bodies and autophagic vacuoles accumulated in hepatocytes of some ...
Adam Shardlow and colleagues investigate whether using cystatin C in addition to serum creatinine can improve diagnosis and risk prediction for progression of CKD among patients in primary care, and estimate the costs of additional testing and monitoring.
Adam Shardlow and colleagues investigate whether using cystatin C in addition to serum creatinine can improve diagnosis and risk prediction for progression of CKD among patients in primary care, and estimate the costs of additional testing and monitoring.
Information on black lung disease (or coal workers pneumoconiosis); how to get a diagnosis for the disease and file a claim under state workers comp systems and the federal black lung program; and benefits available to current and former coal miners with black lung, as well as their surviving spouses and children.
In dystonia, the involuntary abnormal movements cause a driving handicap and a change of the quality of life. A particular shape of dystonia, the Myoclonus Dystonia, is characterized by the ascendancy of myoclonias (abrupt and brief movements) associated with the abnormal dystonia. Myoclonus is an additional source of handicap in the movements of the everyday life, because they distort the precision of movements. Response to oral medications may be incomplete and the tolerance poor, such that deep brain stimulation (DBS) surgery is useful for the major forms but it is also an invasive therapeutics which the operating risk is not totally estimated in the absence of controlled study. Therefore, it is necessary to investigate other pharmacological therapeutic tracks which present a good ratio profit / risk.. Zonisamide is usually used in France in the epilepsys treatment. It showed its efficiency in the progressive myoclonus epilepsy, not only on the seizure but also on the myoclonia. Therefore, ...
en] Abstract Cystatin C is a low-molecular-weight protein which has been proposed as a marker of renal function that could replace creatinine. Indeed, the concentration of cystatin C is mainly determined by glomerular filtration and is particularly of interest in clinical settings where the relationship between creatinine production and muscle mass impairs the clinical performance of creatinine. Since the last decade, numerous studies have evaluated its potential use in measuring renal function in various populations. More recently, other potential developments for its clinical use have emerged. This review summarises current knowledge about the physiology of cystatin C and about its use as a renal marker, either alone or in equations developed to estimate the glomerular filtration rate. This paper also reviews recent data about the other applications of cystatin C, particularly in cardiology, oncology and clinical pharmacology. Clin Chem Lab Med 2008;46:1664-86 ...
BACKGROUND: Human cystatin C is a cysteine protease inhibitor produced by all nucleated cells in the body and the protein is present in all body fluids. The concentration in cerebrospinal fluid (CSF) is considerably higher than in plasma. Cystatin C levels seem to influence the development of Alzheimer disease (AD) and low levels in the brain are associated with an increased risk for AD. The aim of this study was to develop a high throughput assay for the quantification of cystatin C in CSF. METHODS: Antigen excess, imprecision, interference, linearity, recovery, sample stability and reference values were evaluated on Architect ci8200 (Abbott Laboratories, Abbott Park, IL, USA). RESULTS: The assay had an antigen-excess limit at 23 mg/L and was linear over the range of 0.84 to 8.33 mg/L. Results , 8.33 mg/L were automatically rerun in a higher dilution. Within-run coefficient of variation (CV) was 1.71, 1.10 and 0.79%, between day CV was 1.71, 0.39 and 1.45%, between-run CV was 0.58, 0.66 and ...
Methods Cross-sectional analysis of 5386 participants from The Irish Longitudinal Study on Ageing, a cluster-sampled national cohort of community-dwelling adults aged ≥50 years. Cystatin C and creatinine were measured simultaneously using standardised assays. Using generalised additive models, we modelled the distributions of creatinine and cystatin C per year of age from four distributional parameters: location, dispersion, skewness, kurtosis. Among participants with CKD stage 3a, we estimated the predicted probability of cystatin C eGFR ,60 mL/min/1.73 m2 (confirmed CKD) as a function of age. ...
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Definition The word myoclonus ("myo" / muscle, "clonus" / jerk) refers to a brief involuntary twitching of a muscle...
Neuron-Spectrum-5; Neuron-Spectrum-4/EPM; Neuron-Spectrum-AM; Neuromonitor; Neuron-Spectrum-4/P; Neuron-Spectrum-4; Neuron-Spectrum-3; Neuron-Spectrum-2; Neuron-Spectrum-1; Neuron-Spectrum-1/BFB
Genetics Home Reference : 25 Lafora progressive myoclonus epilepsy is a brain disorder characterized by recurrent seizures (epilepsy) and a decline in intellectual function. The signs and symptoms of the disorder usually appear in late childhood or adolescence and worsen with time. Myoclonus is a term used to describe episodes of sudden, involuntary muscle jerking or twitching that can affect part of the body or the entire body. Myoclonus can occur when an affected person is at rest, and it is made worse by motion, excitement, or flashing light (photic stimulation). In the later stages of Lafora progressive myoclonus epilepsy, myoclonus often occurs continuously and affects the entire body. Several types of seizures commonly occur in people with Lafora progressive myoclonus epilepsy. Generalized tonic-clonic seizures (also known as grand mal seizures) affect the entire body, causing muscle rigidity, convulsions, and loss of consciousness. Affected individuals may also experience occipital ...
Background: The L68Q variant of cystatin C is highly amyloidogenic forming aggregates in individuals with HCCAA. Results: Spermatozoa from mice expressing human L68Q cystatin C exhibit fertility defects and increased levels of amyloid. Conclusion: L68Q epididymal fluid containing cystatin C amyloid is harmful for sperm function. Significance: Amyloid in the reproductive tract may contribute to male factor infertility. Hereditary cystatin C amyloid angiopathy is an autosomal dominant disorder in which a variant form of cystatin C (L68Q) readily forms amyloid deposits in cerebral arteries in affected individuals resulting in early death. L68Q protein deposits in human cystatin C amyloid angiopathy patients have also been found in tissues outside of the brain including the testis, suggesting possible effects on fertility. Heterozygous transgenic mice (L68Q) that express the human L68Q variant of cystatin C under the control of the mouse cystatin C promoter were unable to generate offspring, ...
A partial cystatin cDNA from rainbow trout was generated by reverse transcription polymerase chain reaction with two degenerate primers. The partial cystatin PCR product was 168 bp and used to screen trout liver λgt 11 cDNA library. Four positive clones were isolated and designated as cstl, cst2, cst3 and cst4. Only cst2 contained the full-length cystatin cDNA which was 674 bp and included 5 untranslated region and the polyadenylation signal sequence AATAAA in the 3 region. Translation of the cDNA contains 132 amino acid residues. Comparison of the amino acid sequence with those of family II cystatin indicated that the 21 amino acids at N-terminal end is a signal peptide that leads to cystatin secretion, and the 111 amino acids are mature cystatin. Four cysteine residues in the cystatin may form two disulfide bonds for the secondary structure. Cst2 was subcloned into pGEM-3z for Northern and Southern blot experiments. Northern blot indicated that trout cystatin mRNA is about 750 bp. Cystatin ...
Crompton and colleagues recently published the clinical and genetic description of a large family with Familial Adult Myoclonic Epilepsy (FAME). This phenotype is particularly interesting since it provides some insight into how neurologists conceptualize twitches and jerks. It is also a good example that large families do not necessarily result in a narrow linkage region, particularly when centromeric regions are involved.. What is myoclonus? Despite usually mentioned in the context of epilepsy, most people are inherently familiar with myoclonus. Most of us "twitch" when we fall asleep and sometimes experience this twitch as part of a dream. These episodes are entirely normal and are called hypnic jerks, but they give people a good idea of what a sudden, brief, shocklike, involuntary movement caused by muscular contraction or inhibition would feel like. Myoclonus in the setting of epilepsy is usually mentioned as part of a Juvenile Myoclonic Epilepsy (JME) or Progressive Myoclonus Epilepsy ...
We found that higher cystatin C concentrations showed a greater association with severe CMB pathology especially in patients of the highest quartile. One standard deviation increase in the log-transformation of cystatin C levels also showed a closer relationship with severe CMBs. The estimated GFR and microalbumin/creatinine ratio failed to show a significant association with the number of CMBs. Moreover, the association was sustained in patients with deep or infratentorial CMBs but not among those with strictly lobar CMBs. In addition, the correlation between the CMB grades and cystatin C in patients with lacunar stroke showed a stronger association than that in patients with non lacunar stroke. Cystatin C was the most powerful indicator for CMBs among the three renal markers. Our results corroborate previous studies showing that renal dysfunction is independently associated with CMBs [8, 29]. Furthermore, we showed that cystatin C may reflect the severity of CMBs in a dose dependent manner ...
IFN-γ is so far the only cytokine able to induce by its own the synthesis of iNOS and the release of NO from MPM. However, it has been shown recently that IFN-γ-induced TNF-α is a prerequisite for in vitro production of NO released by MPM [2,4]. In addition to TNF-α, many other cytokines and bacterial products including transforming growth factor β, IL-10, TNF-α/β, GM-CSF and LPS [5-8,13,16,22,25] can also trigger NO production by acting in synergistic pairs on IFN-γ-activated MPM. We recently demonstrated that representative members of the cystatin superfamily, and particularly CC, can stimulate the release of NO from IFN-γ-activated MPM by stimulating the iNOS/NO system [12]. The results reported here show that CC stimulates the synthesis of TNF-α and IL-10 and upregulates the NO production by IFN-γ-activated MPM. The early iNOS induction by IFN-γ, followed by a CC stimulation, leads to maximal production of NO by MPM. This suggests that CC acts as an amplifier, but only if the ...
Renal dysfunction is associated with increased cardiovascular morbidity and mortality. The aim of this cross-sectional study was to investigate the relationship between the glomerular filtration marker cystatin C and other cardiovascular risk markers and morbidity in elderly males. Cystatin C was measured in a group of 77-year-old males (n=792) and compared cystatin C with other known risk markers for cardiovascular disease. Cystatin C values were significantly increased in individuals with diabetes (p=0.05) and cardiovascular diseases (p,0.0001). There were significant correlations between cystatin C values and body mass index, HbA1c, insulin, triglycerides and hsCRP.. ...
Lafora disease (LD) is an autosomal recessive late onset, progressive myoclonic epilepsy with a high prevalence in the miniature Wirehaired Dachshund. The disease is due to a mutation in the Epm2b gene which results in intracellular accumulation of abnormal glycogen (Lafora bodies)... A characteristic feature of the disease is spontaneous and reflex myoclonus; however clinical signs and disease progression are not well described... Average age of onset of clinical signs was 6.94 years (3.5-12). The most common initial presenting sign was reflex and spontaneous myoclonus (77.8%). Other presenting signs included hypnic myoclonus (51.9%) and generalized seizures (40.7%). Less common presenting signs include focal seizures, "jaw smacking", "fly catching", "panic attacks", impaired vision, aggression and urinary incontinence. All these clinical signs may appear, and then increase in frequency and intensity over time. The myoclonus in particular becomes more severe and more refractory to treatment. ...
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A medication similar to Marquis (the only approved medication to treat EPM), but in a top dress pellet form will be introduced in early December. Both Marquis and this new medication(generic name diclazuril), prevent the EPM protozoa from growing and replicating. Neither actually seem to kill the EPM organism. However, the bodys own immune system can kill the protozoa once it stops multiplying. Dr. Tom Divers, of Cornell Veterinary School, presented the information at a recent seminar. Dr. Divers explained that diclazuril more easily crosses the blood brain barrier than ponazuril, the active ingredient in Marquis. Both medications have a wide margin of safety. Diclazuril has been used to treat coccidial infections in other animals. The other class of medication used to treat EPM is pyrimethamine which actually kills the EPM organism. However, there are cases where the horse gets worse when treated with the pryimethamine as the protozoa die off and there is an inflammatory response ...
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What is cystatin C amyloidosis (ACys)?What is cystatin C amyloidosis (ACys)?

... is cystatin C, which is a cysteine protease inhibitor that contains a point mutation. This condition is clinically termed HCHWA ... The precursor protein in cystatin C amyloidosis (ACys) ... What is cystatin C amyloidosis (ACys)?. Updated: May 09, 2019 * ... encoded search term (What is cystatin C amyloidosis (ACys)?) and What is cystatin C amyloidosis (ACys)? What to Read Next on ... The precursor protein in cystatin C amyloidosis (ACys) is cystatin C, which is a cysteine protease inhibitor that contains a ...
more infohttps://www.medscape.com/answers/335414-106004/what-is-cystatin-c-amyloidosis-acys

Cystatin - WikipediaCystatin - Wikipedia

... cystatin C, a marker of kidney function), CST4, CST5, CST6, CST7, CST8, CST9, CST11, CSTA (cystatin A), CSTB (cystatin B)[ ... Chicken cystatin quickly passed the membrane of MCF-10A neo T cells and inhibited cathepsin B when it was acylated with fatty ... Cystatin: a protein that flips out! QUite Interesting PDB Structure article at PDBe Lee C, Bongcam-Rudloff E, Sollner C, Jahnen ... These are cystatin-like proteins found in a range of organisms: plant phytocystatins, fetuin in mammals, insect cystatins, and ...
more infohttps://en.wikipedia.org/wiki/Cystatin

Cystatin B
     Summary Report | CureHunterCystatin B Summary Report | CureHunter

An intracellular cystatin subtype that is found in a broad variety of cell types. It is a cytosolic enzyme inhibitor that ... Cystatin B. Subscribe to New Research on Cystatin B An intracellular cystatin subtype that is found in a broad variety of cell ... caused by mutations in the cystatin B gene and in the cystatin B-deficient (Cstb-/-) mouse model and to validate imaging ... a translational diffusion-tensor imaging study in patients and cystatin B-deficient mice.". 12/01/2013 - "Cystatin B (CSTB) is ...
more infohttp://www.curehunter.com/public/keywordSummaryD055313-Cystatin-B.do

Human Cystatin B / CSTB ELISA Pair Set | ABIN2010313Human Cystatin B / CSTB ELISA Pair Set | ABIN2010313

Find and order accessory reagents and products like Human Cystatin B / CSTB ELISA Pair Set on www.antibodies-online.com. Order ... Human Cystatin B / CSTB ELISA Pair Set Human Cystatin B / CSTB ELISA Pair Set. Details for Product No. ABIN2010313, Supplier: ... The Human Cystatin B / CSTB ELISA Pair Set is for the quantitative determination of Human Cystatin B / CSTB.This ELISA Pair Set ... More product categories related to Cystatin B (Stefin B) Accessory Reagent * 234 anti-Cystatin B (Stefin B) Primary Antibodies ...
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Determination of cerebrospinal fluid cystatin C on Architect ci8200Determination of cerebrospinal fluid cystatin C on Architect ci8200

BACKGROUND: Human cystatin C is a cysteine protease inhibitor produced by all nucleated cells in the body and the protein is ... Cystatin C levels seem to influence the development of Alzheimer disease (AD) and low levels in the brain are associated with ... Cystatin C, Cerebrospinal fluid, Alzheimers disease National Category Medical and Health Sciences Identifiers. URN: urn:nbn:se ... The aim of this study was to develop a high throughput assay for the quantification of cystatin C in CSF. METHODS: Antigen ...
more infohttp://uu.diva-portal.org/smash/record.jsf?pid=diva2:358263

Molecular cloning and sequence analysis of cystatin from rainbow trout (Oncorhynchus mykiss)Molecular cloning and sequence analysis of cystatin from rainbow trout (Oncorhynchus mykiss)

Northern blot indicated that trout cystatin mRNA is about 750 bp. Cystatin is expressed in all tissues examined but at various ... and the 111 amino acids are mature cystatin. Four cysteine residues in the cystatin may form two disulfide bonds for the ... The partial cystatin PCR product was 168 bp and used to screen trout liver λgt 11 cDNA library. Four positive clones were ... Comparison of the amino acid sequence with those of family II cystatin indicated that the 21 amino acids at N-terminal end is a ...
more infohttps://ir.library.oregonstate.edu/concern/graduate_thesis_or_dissertations/73666801v

Cystatin C: current position and future prospects. - Séronie-Vivien SophieCystatin C: current position and future prospects. - Séronie-Vivien Sophie

en] Abstract Cystatin C is a low-molecular-weight protein which has been proposed as a marker of renal function that could ... This review summarises current knowledge about the physiology of cystatin C and about its use as a renal marker, either alone ... Indeed, the concentration of cystatin C is mainly determined by glomerular filtration and is particularly of interest in ... This paper also reviews recent data about the other applications of cystatin C, particularly in cardiology, oncology and ...
more infohttp://orbi.ulg.ac.be/handle/2268/4281

Serum Cystatin C Predicts Mortality With Cirrhotic Ascites | Physicians Weekly for Medical News, Journals & ArticlesSerum Cystatin C Predicts Mortality With Cirrhotic Ascites | Physician's Weekly for Medical News, Journals & Articles

Serum Cystatin C Predicts Mortality With Cirrhotic Ascites by Physicians Weekly , Sep 22, 2017 , 0 comments ... "Predictive models based on serum cystatin C level instead of serum creatinine level would be more helpful in the assessment of ... The researchers found that serum creatinine and cystatin C levels were 0.9 and 1.1 mg/L, respectively. International normalized ... 22, 2017 (HealthDay News) - Serum cystatin C level is an excellent predictor of mortality in patients with cirrhotic ascites, ...
more infohttps://www.physiciansweekly.com/serum-cystatin-c-predicts-mortality-with-cirrhotic-ascites/

Serum cystatin C is associated with other cardiovascular risk markers and cardiovascular disease in elderly menSerum cystatin C is associated with other cardiovascular risk markers and cardiovascular disease in elderly men

Cystatin C was measured in a group of 77-year-old males (n=792) and compared cystatin C with other known risk markers for ... Serum cystatin C is associated with other cardiovascular risk markers and cardiovascular disease in elderly men. Larsson, ... Cystatin C values were significantly increased in individuals with diabetes (p=0.05) and cardiovascular diseases (p,0.0001). ... Cardiovascular disease, Cystatin C, Diabetes mellitus, Glomerular filtration rate, Kidney, Human National Category Medical and ...
more infohttp://uu.diva-portal.org/smash/record.jsf?pid=diva2:126715

Cystatin C and Estimated Glomerular Filtration Rate as Predictors for Adverse Outcome in Patients With ST-Elevation and non-ST...Cystatin C and Estimated Glomerular Filtration Rate as Predictors for Adverse Outcome in Patients With ST-Elevation and non-ST...

The median cystatin C value was 0.83 mg/L. Increasing quartiles of cystatin C were strongly associated with death and MI (6.9 ... cystatin C) to 0.6941 (CKD-EPI). Neither cystatin C nor other creatinine-based equations increased the area under the curve ... Cystatin C and Estimated Glomerular Filtration Rate as Predictors for Adverse Outcome in Patients With ST-Elevation and non-ST- ... YOU ARE HERE: Home , Latest in Cardiology , Cystatin C and Estimated Glomerular Filtration Rate as Predictors for Adverse ...
more infohttp://www.acc.org/latest-in-cardiology/journal-scans/2011/12/20/17/22/cystatin-c-and-estimated-glomerular-filtration-rate-as-predictors

Examining the utility of cystatin C as a confirmatory test of chronic kidney disease across the age range in middle-aged and...Examining the utility of cystatin C as a confirmatory test of chronic kidney disease across the age range in middle-aged and...

We observed progressive variability in cystatin C with increasing age. Compared with creatinine, cystatin C levels rose sharply ... Background Cystatin C has been proposed as a confirmatory test of chronic kidney disease (CKD). This is most applicable to ... Examining the utility of cystatin C as a confirmatory test of chronic kidney disease across the age range in middle-aged and ... Examining the utility of cystatin C as a confirmatory test of chronic kidney disease across the age range in middle-aged and ...
more infohttp://jech.bmj.com/content/early/2018/01/13/jech-2017-209864

The Cystatin S gene maps to rat Chromosome 3, to which D1mgh18 is re-assigned from Chromosome 1<...The Cystatin S gene maps to rat Chromosome 3, to which D1mgh18 is re-assigned from Chromosome 1<...

The Cystatin S gene maps to rat Chromosome 3, to which D1mgh18 is re-assigned from Chromosome 1. Mammalian Genome. 1997 Dec;8( ... The Cystatin S gene maps to rat Chromosome 3, to which D1mgh18 is re-assigned from Chromosome 1. / Duran Alonso, M. Beatriz; ... title = "The Cystatin S gene maps to rat Chromosome 3, to which D1mgh18 is re-assigned from Chromosome 1", ... T1 - The Cystatin S gene maps to rat Chromosome 3, to which D1mgh18 is re-assigned from Chromosome 1 ...
more infohttps://jhu.pure.elsevier.com/en/publications/the-cystatin-s-gene-maps-to-rat-chromosome-3-to-which-d1mgh18-is--3

Cystatin-BCystatin-B

The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active ... Cystatin B is a progression marker of human epithelial ovarian tumors mediated by the TGF-? signaling pathway.. ... A role for disease-causing mutations in cystatin B gene in patients with juvenile myoclonic epilepsy was not supported.. ... Cystatin B as a potential diagnostic biomarker in ovarian clear cell carcinoma.. ...
more infohttps://pharos.nih.gov/idg/targets/P04080

Goat Anti-Human Cystatin B / Stefin B, (C Terminus) [ER-14-0390]Goat Anti-Human Cystatin B / Stefin B, (C Terminus) [ER-14-0390]

SpecificityC TerminusStorage/StabilityAliquot and store at -20°C Minimize freezing and thawing More InformationImmunogenThe immunogen was a 13-residue peptide matching a sequence from the C Terminus of Human CSTB See Accession Number s NP_000091 1 Formulation 0 5 mg/ml in TBS 0 02% NaNH3 pH7
more infohttps://www.raybiotech.com/goat-anti-human-cystatin-b-stefin-b-c-terminus/

Fertility Defects in Mice Expressing the L68Q Variant of Human Cystatin C A ROLE FOR AMYLOID IN MALE INFERTILITY - Lund...Fertility Defects in Mice Expressing the L68Q Variant of Human Cystatin C A ROLE FOR AMYLOID IN MALE INFERTILITY - Lund...

Hereditary cystatin C amyloid angiopathy is an autosomal dominant disorder in which a variant form of cystatin C (L68Q) readily ... Heterozygous transgenic mice (L68Q) that express the human L68Q variant of cystatin C under the control of the mouse cystatin C ... Fertility Defects in Mice Expressing the L68Q Variant of Human Cystatin C A ROLE FOR AMYLOID IN MALE INFERTILITY. Research ... L68Q protein deposits in human cystatin C amyloid angiopathy patients have also been found in tissues outside of the brain ...
more infohttps://portal.research.lu.se/portal/en/publications/fertility-defects-in-mice-expressing-the-l68q-variant-of-human-cystatin-c-a-role-for-amyloid-in-male-infertility

Gentaur Molecular :Ray Biotech \ Recombinant Human Cystatin C \ 228-10301-3Gentaur Molecular :Ray Biotech \ Recombinant Human Cystatin C \ 228-10301-3

Recombinant Human Cystatin C \ 228-10301-3 for more molecular products just contact us ... The Cystatin-C amino acid sequence is identical to UniProtKB/Swiss-Prot entry Q6FGW9 amino acids 28 146.. The Cystatin-C is ... Index / Ray Biotech / Recombinant Human Cystatin C / Product Detail : 228-10301-3 Recombinant Human Cystatin C. Related ... Cystatin-C, Cystatin-3, Neuroendocrine basic polypeptide, Gamma-trace, Post-gamma-globulin, CST3, MGC117328. ...
more infohttp://www.antibody-antibodies.com/product_det.php?id=1783531&supplier=search&name=Recombinant%20Human%20Cystatin%20C

Gentaur Molecular :Prospecbio \ Mouse Anti Human Cystatin B CSTB \ ant-339Gentaur Molecular :Prospecbio \ Mouse Anti Human Cystatin B CSTB \ ant-339

Mouse Anti Human Cystatin B CSTB \ ant-339 for more molecular products just contact us ... Index / Prospecbio / Mouse Anti Human Cystatin B CSTB / Product Detail : ant-339 Mouse Anti Human Cystatin B CSTB. Related ... We have also other products like : Mouse Anti Human Cystatin B CSTB. Related products : Mouse Anti Human Cystatin B CSTB ... Mouse Anti Human Cystatin B CSTB antibody storage GENTAUR recommends for long therm storage to freeze at -24 C. For short time ...
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Cystatin C-based equations: dont repeat the same errors with analytical considerations. - Delanaye PierreCystatin C-based equations: don't repeat the same errors with analytical considerations. - Delanaye Pierre

Reference : Cystatin C-based equations: dont repeat the same errors with analytical considerations.. ... Cystatin C-based equations: dont repeat the same errors with analytical considerations. ... en] Blood Chemical Analysis/methods ; Creatinine/blood ; Cystatin C ; Cystatins/blood ; Enzyme-Linked Immunosorbent Assay ; ...
more infohttps://orbi.uliege.be/handle/2268/4785

Cystatin C, a novel indicator of renal function, reflects severity of cerebral microbleeds | BMC Neurology | Full TextCystatin C, a novel indicator of renal function, reflects severity of cerebral microbleeds | BMC Neurology | Full Text

... cystatin C and estimated GFR, r = 0.43, p , 0.01; cystatin C and microalbumin/creatinine ratio, r = 0.23, p , 0.01; cystatin C ... Cystatin C was a significant indicator of deep or infratenorial CMBs, but not strictly lobar CMBs. In addition, cystatin C ... Compared to patients with the lowest levels of cystatin C, patients with high cystatin C had a more significant association ... In models including both quartiles of cystatin C and estimated GFR, only cystatin C quartiles were significant (the highest vs ...
more infohttps://bmcneurol.biomedcentral.com/articles/10.1186/1471-2377-14-127

A beta Interaction with Transthyretin and Human cystatin C - The University of Sheffield Kaltura Digital Media HubA beta Interaction with Transthyretin and Human cystatin C - The University of Sheffield Kaltura Digital Media Hub

A beta Interaction with Transthyretin and Human cystatin C From batchUser on May 8th, 2017 ... Co-deposition of other amyloidogenic proteins like transthyretin, human cystatin C have been found in the plaques along with A ...
more infohttps://digitalmedia.sheffield.ac.uk/media/A+beta+Interaction+with+Transthyretin+and+Human+cystatin+C/0_72t59meu/69889811

CSTA monoclonal antibody (M14), clone 4D8 - (H00001475-M14) - Products - AbnovaCSTA monoclonal antibody (M14), clone 4D8 - (H00001475-M14) - Products - Abnova

The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active ...
more infohttp://www.abnova.com/products/products_detail.asp?catalog_id=H00001475-M14

ORBi: Browsing ORBiORBi: Browsing ORBi

GFR Estimation Using Standardized Cystatin C in Kidney Transplant Recipients. Masson, Ingrid; Maillard, Nicolas; Tack, Ivan et ... Background: The utility of serum cystatin C (SCysC) as a filtration marker in kidney transplantation is uncertain. We took ... Background: The utility of serum cystatin C (SCysC) as a filtration marker in kidney transplantation is uncertain. We took ...
more infohttp://orbi.ulg.ac.be/browse?type=journal&value=American+Journal+of+Kidney+Diseases

CYSTEINE PROTEINASE INHIBITOR LEVEL IN TUMOR AND NORMAL TISSUES IN CONTROL AND CURED MICECYSTEINE PROTEINASE INHIBITOR LEVEL IN TUMOR AND NORMAL TISSUES IN CONTROL AND CURED MICE

... assay The cystatin C ELISA test is a sandwich immunoassay, which uses immobilized rabbit antihuman cystatin C Pab and mouse ... increase in cystatin C. Obviously, the decrease in cystatin C concentration predominantly in tumor tissue was connected with ... by low cystatin C concentrations in tumor tissues and decreased cystatin C concentrations (to a lesser degree) were also ... Summary: Cystatin C is the best known extracellular endogenous cysteine proteinase inhibitor and has been studied as a possible ...
more infohttp://ukrain.ua/statti/171.htm

Cystatin CCystatin C

A cystatin C test may be used to screen for and monitor kidney dysfunction in special cases. ... Cystatin C is a relatively small protein that is filtered from the blood by the kidneys and broken down at a constant rate. ... Because cystatin C levels fluctuate with changes in GFR, there has been interest in the cystatin C test as one method of ... Lowry, F. (2009 February 27). Cystatin C, High Levels of Serum Cystatin C and Chronic Kidney Disease Linked to Age-Related ...
more infohttps://labtestsonline.org/tests/cystatin-c
  • citation needed] The cystatin family includes: The Type 1 cystatins, which are intracellular and are present in the cytosol of many cell types, but can also appear in body fluids at significant concentrations. (wikipedia.org)
  • Cystatin C has been shown to be a more sensitive renal indicator than conventional renal markers. (biomedcentral.com)
  • Under the assumption that similar pathologic mechanisms of the small vessel exist in the brain and kidney, we hypothesized that the levels of cystatin C may delineate the relationship between CMBs and renal insufficiency by detecting subclinical kidney dysfunction, which may be underestimated by other indicators, and thus reflect the severity of CMBs more accurately. (biomedcentral.com)
  • Cystatin C may be the most sensitive indicator of CMB severity among the renal disease markers. (biomedcentral.com)
  • Background: The L68Q variant of cystatin C is highly amyloidogenic forming aggregates in individuals with HCCAA. (lu.se)
  • Heterozygous transgenic mice (L68Q) that express the human L68Q variant of cystatin C under the control of the mouse cystatin C promoter were unable to generate offspring, suggesting the presence of L68Q cystatin C amyloid affected sperm function. (lu.se)
  • Hereditary cystatin C amyloid angiopathy is an autosomal dominant disorder in which a variant form of cystatin C (L68Q) readily forms amyloid deposits in cerebral arteries in affected individuals resulting in early death. (lu.se)
  • Results: Spermatozoa from mice expressing human L68Q cystatin C exhibit fertility defects and increased levels of amyloid. (lu.se)
  • Conclusion: L68Q epididymal fluid containing cystatin C amyloid is harmful for sperm function. (lu.se)
  • Examination of the epididymal fluid and spermatozoa from L68Q mice showed increased levels and distinct forms of cystatin C amyloid that were not present in WT mice. (lu.se)
  • Comparison of the amino acid sequence with those of family II cystatin indicated that the 21 amino acids at N-terminal end is a signal peptide that leads to cystatin secretion, and the 111 amino acids are mature cystatin. (oregonstate.edu)
  • Cystatin is expressed in all tissues examined but at various levels. (oregonstate.edu)
  • International normalized ratio (INR) and serum bilirubin, sodium, and cystatin C levels were independent predictors of mortality, in multivariate analyses, while INR and serum sodium and cystatin C levels were independent predictors of the development of type 1 hepatorenal syndrome (HRS-1). (physiciansweekly.com)
  • FRIDAY, Sept. 22, 2017 (HealthDay News) - Serum cystatin C level is an excellent predictor of mortality in patients with cirrhotic ascites, according to a study published online Sept. 14 in the Journal of Gastroenterology and Hepatology . (physiciansweekly.com)
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