Knowledgebase protein acyl coa dehydrogenases. Medical search. Frequent questions

We examined the enzyme protein and biosynthesis of human trifunctional protein harboring enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase activity in cultured skin fibroblasts from two patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (rhea-db.org)
Taken together, the results obtained show that in both patients, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is caused by an abnormality in the trifunctional protein, even though there is a heterogeneity in both patients. (rhea-db.org)
[4] Trifunctional protein deficiency is characterized by decreased activity of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), long-chain enoyl-CoA hydratase, and long-chain thiolase. (wikidoc.org)

Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). (wikipedia.org)

This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. (wikipedia.org)
Mitochondrial trifunctional protein deficiency. (rhea-db.org)
Trifunctional enzyme subunit beta, mitochondrial (TP-beta) also known as 3-ketoacyl-CoA thiolase , acetyl-CoA acyltransferase , or beta-ketothiolase is an enzyme that in humans is encoded by the HADHB gene . (wikidoc.org)
HADHB is a subunit of the mitochondrial trifunctional protein and has thiolase activity. (wikidoc.org)
This gene encodes the beta subunit of the mitochondrial trifunctional protein, a catalyst of mitochondrial beta-oxidation of long chain fatty acids . (wikidoc.org)
The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. (wikidoc.org)

Search proteins in UniProtKB for this molecule. (rhea-db.org)
The HADHB protein catalyzes the final step of beta-oxidation, in which 3-ketoacyl CoA is cleaved by the thiol group of another molecule of Coenzyme A . The thiol is inserted between C-2 and C-3, which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons shorter. (wikidoc.org)
The association of a protein and a molecule can be determined from their co-occurrences in biomedical abstracts. (biomedcentral.com)
Extensive semantically related co-occurrences of a protein's name and a molecule's name in the sentences of biomedical abstracts can be considered as indicative of the association between the protein and the molecule. (biomedcentral.com)
It automatically extracts each co-occurrence of a protein-molecule pair that represents semantic relationship between the pair. (biomedcentral.com)
Towards this, we present novel semantic rules that identify the semantic relationship between each co-occurrence of a protein-molecule pair using the syntactic structures of sentences and linguistics theories. (biomedcentral.com)

The final result of beta-oxidation is acetyl-CoA. (wikipedia.org)
As short-chain acyl-CoA dehydrogenase is involved in beta-oxidation, a deficiency in this enzyme is marked by an increased amount of fatty acids. (wikipedia.org)

The ACADS gene associated with short-chain acyl-coenzyme A dehydrogenase deficiency. (wikipedia.org)
this is also known as butyryl-CoA dehydrogenase deficiency. (wikipedia.org)
Outcomes in pediatric studies of medium-chain acyl-coA dehydrogenase (MCAD) deficiency and phenylketonuria (PKU): a review. (cdc.gov)
Mutations in this gene, along with mutations in HADHA , result in trifunctional protein deficiency . (wikidoc.org)

References to protein records in UniProtKB are made through entries in the ProForm sub-ontology within PRO (Figure 1 ). (biomedcentral.com)

Acyl-CoA dehydrogenase, C-2 to C-3 short chain is an enzyme that in humans is encoded by the ACADS gene. (wikipedia.org)
ProComp leverages, and cross references, entries in existing protein-centric informatics resources, including the protein complexes that are represented in the Cellular Component branch of the Gene Ontology. (biomedcentral.com)

The deprotonated 3S-C8-CoA was clearly located within the active-site cleft of the enzyme. (rcsb.org)

Although existing protin-centric informatics resources provide the biomedical research community with well-curated compendia of protein sequence and structure, these resources lack formal ontological representations of the relationships among the proteins themselves. (biomedcentral.com)

The diminution of trifunctional protein was associated with a decreased activity of enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase, when measured using medium-chain to long-chain substrates. (rhea-db.org)
b) In cells from patient 2, the rate of degradation of newly synthesized trifunctional protein was faster than that in control cells, giving rise to a trifunctional protein amounting to 60% of the control levels. (rhea-db.org)
These data suggest a subtle abnormality of trifunctional protein in cells from patient 2. (rhea-db.org)

The flavoenzyme medium-chain acyl-CoA dehydrogenase (MCAD) eliminates the alpha-proton of the substrate analog, 3-thiaoctanoyl-CoA (3S-C8-CoA), to form a charge-transfer complex with deprotonated 3S-C8-CoA. (rcsb.org)
Comparison of the stability and substrate specificity of purified peroxisomal 3-oxoacyl-CoA thiolases A and B from rat liver. (rhea-db.org)

Aim of this study was to generate 3D models of protein drug targets in Erysipelothrix rhusiopathiae by homology modelling. (ijpsr.com)
Among 4153 proteins reported from Erysipelothrix rhusiopathiae , 396 proteins were identified as potential drug targets. (ijpsr.com)
The modelled proteins were subjected to protein-ligand docking analysis with standard antibiotics such as Ribostamycin, Cefalotin, Pefloxacin, Penicillamine, Artenimol, Cycloserine against their respective drug targets. (ijpsr.com)

JNK activation is required for apoptosis but c-jun, a protein in the JNK signaling pathway, is not always required. (jnksignaling.com)
Because proteins are often functional only as members of stable protein complexes, the PRO Consortium, in collaboration with existing protein and pathway databases, has launched a new initiative to implement logical and consistent representation of protein complexes. (biomedcentral.com)

The aggregation of both amyloid beta (Abeta) peptides extracellularly and Tau proteins intracellularly plays key roles in the pathological consequences of AD, which lead to cholinergic neurodegeneration and eventually death. (inra.fr)

ProEvo represents evolutionary relatedness of proteins. (biomedcentral.com)

Among these antibiotics Ribostamycin was identified as a potent drug against the Protein Disulphide Isomerase with a significant binding energy of -7.35Kcal/mol with formation of 5 hydrogen bonds. (ijpsr.com)

The arrangement between the flavin ring and deprotonated 3S-C8-CoA is consistent with a charge transfer interaction with the negatively charged acyl-chain of 3S-C8-CoA as an electron donor stacking on the pyrimidine moiety of the flavin ring as an electron acceptor. (rcsb.org)

Structure of the transition state analog of medium-chain acyl-CoA dehydrogenase. (rcsb.org)
The 3-hydroxy-acyl-CoA dehydrogenase activity with medium-chain to long-chain substrates was decreased drastically, with minor changes in activities of the two other enzymes. (rhea-db.org)

The encoded protein has 412 amino acids, and its size is 44.3 kDa (Human) or 44.9 KDa (Mouse). (wikipedia.org)

Logical and semantic access to related protein forms is critical for advancing bioinformatics approaches to representing, modeling, and reasoning about complex biological systems at the genomic and cellular level [ 1 ]. (biomedcentral.com)

Representing species-specific proteins and protein complexes in ontologies that are both human- and machine-readable facilitates the retrieval, analysis, and interpretation of genome-scale data sets. (biomedcentral.com)
ProComp, the focus of this manuscript, represents multi-protein complexes, with an initial (but not exclusive) emphasis on protein components of complexes in mouse and human. (biomedcentral.com)
The explicit representation of protein complexes in PRO--defining each member of the complex at the level of its isoform, variant, or modified form--provides the ability to represent complex biological knowledge as it is emerging in the experimental research community in structures that are both human readable and accessible to algorithmic approaches. (biomedcentral.com)

We describe here how the PRO Consortium is meeting the challenge of representing species-specific protein complexes, how protein complex representation in PRO supports annotation of protein complexes and comparative biology, and how PRO is being integrated into existing community bioinformatics resources. (biomedcentral.com)
A major contribution of PRO as a protein biology community informatics resource is that it provides a formal ontological structure with foundation in Basic Formal Ontology http://www.ifomis.org/bfo/ to describe types of protein complexes and gives these types unique, permanent identifiers http://www.obofoundry.org/id-policy.shtml . (biomedcentral.com)

The encoded protein can also bind RNA and decreases the stability of some mRNAs . (wikidoc.org)

PPFBM's performance over these system increases steadily as the number of training protein increases. (biomedcentral.com)

The homology model of the proteins generated in this study can be exploited in further research using computational drug discovery and design to accelerate the research on disease management and pathogen control of Erysipelothrix rhusiopathiae induced swine erysipelas. (ijpsr.com)

First, it determines the set S r of annotated proteins that is semantically similar to p by matching the molecules representing p and the annotated proteins. (biomedcentral.com)

The experimental results demonstrated that PPFBM outperforms other systems that predict protein function from the textual information found within biomedical abstracts. (biomedcentral.com)

ProForm represents species-specific and species-independent classes of protein isoforms, co- and post-translationally modified forms, and variant forms. (biomedcentral.com)
PPFBM represents each protein by the other molecules that associate with it in the abstracts referenced in the protein's entries in reliable biological databases. (biomedcentral.com)

Along these lines, it has been shown that overexpression of mig-6 in Rat2 fibroblasts leads to activation of retinoblastoma resulting in a cell cycle arrest.24 Notably, we found elevated levels of the activator protein-1 transcription factor c-Jun in mig-6 knockout livers after PH. (jnksignaling.com)

START domain-containing proteins (STARDs) are responsible for lipid metabolism. (medsci.org)

The large stabilization energy by charge transfer probably plays an important role in determining the alignment of the flavin ring with 3S-C8-CoA. (rcsb.org)

These associated molecules are highly predictive of the potential functions of proteins. (biomedcentral.com)

PRO is a unique database resource for species-specific protein complexes. (biomedcentral.com)
A demo of PPFBM that annotates each input Yeast protein (SGD (Saccharomyces Genome Database). (biomedcentral.com)

Frequent loss of mutation-specific mismatch repair protein expression in nonneoplastic endometrium of Lynch syndrome patients. (cdc.gov)
[10] Additionally, HADHB has been shown to bind to the distal 3' untranslated region of renin mRNA, thereby regulating renin protein expression. (wikidoc.org)

SCAD is specific to short-chain fatty acids, between C2 and C3-acylCoA. (wikipedia.org)

The crystalline complex was obtained by co-crystallizing MCAD in the oxidized form with 3S-C8-CoA. (rcsb.org)

That is, PPFBM's prediction performance becomes more accurate constantly, as the size of training proteins gets larger. (biomedcentral.com)

been implicated in the negative regulation of EGFR signaling23 and such proteins eventually account for EGFR regulation at later time points during liver regeneration. (jnksignaling.com)
Protein complexes are distinguished from protein-protein interactions in that they are continuant entities, i.e. they endure or continue to exist through time. (biomedcentral.com)
This is because every time a new set of test proteins is added to the current set of training proteins. (biomedcentral.com)

Anatomy3

Organisms2

Diseases2

Chemicals and Drugs77

Analytical, Diagnostic and Therapeutic Techniques and Equipment3

Phenomena and Processes12

Disciplines and Occupations4

Information Science17

Health Care1

Long-chain 3-hydro3

Cardiac Organellar Protein Atlas Knowledgebase1

Mitochondrial6

Molecule6

Beta-oxidation2

Deficiency4

UniProtKB1

Gene2

Enzyme1

Sequence1

Trifunctional protein3

Substrate2

Drug Targets3

Pathway2

Peptides1

Evolutionary1

Isomerase1

Consistent1

Medium-chain2

Amino acids1

Semantic1

Human3

Biology2

Stability1

Increases1

Disease1

Similar1

Biomedical1

Represents2

Levels1

Responsible1

Charge1

Potential1

Database2

Expression2

Specific1

Form1

Size1

Time3