The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. (nih.gov)
Defects in this gene that affect the peroxisomal fatty acid beta-oxidation activity are a cause of D-bifunctional protein deficiency (DBPD). (nih.gov)
Another major function of the peroxisomal beta-oxidation system is related to the biosynthesis of polyunsaturated fatty acid (C22:6w3). (medscape.com)
DBPD is a disorder of peroxisomal fatty acid beta-oxidation. (mitosciences.com)
The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. (utsouthwestern.edu)
b Identification of putative peroxisomal enzymes and enyzmes of mitochondrial beta-oxidation. (biomedcentral.com)
Peroxisomes are intracellular organelles that contain enzymes for beta-oxidation. (msdmanuals.com)
Involved in the initial and rate-limiting step of peroxisomal beta-oxidation of straight-chain saturated and unsaturated very-long-chain fatty acids (PubMed:7876265, PubMed:15060085, PubMed:17458872, PubMed:17603022, PubMed:32169171, PubMed:33234382). (swisspalm.org)
Biosynthesis2
Although most xyloglucans (XyGs) biosynthesis enzymes have been identified, the molecular mechanism that defines XyG branching patterns is unclear. (bvsalud.org)
He enjoys an international reputation for his research on phospholipases A, cardiolipin biosynthesis in eukaryotes, lysophospholipases, phosphatidylcholine biosynthesis for lung surfactant, plasmalogen biosynthesis in peroxisomes, diagnosis of peroxisomal disorders and most recently his work on alkyl-dihydroxyacetone phosphate synthase. (ornine.best)
Dehydrogenase5
The D-bifunctional protein is composed of three enzymatic domains: the N-terminal short chain alcohol dehydrogenase reductase (SDR), central hydratase domain, and the C-terminal sterol carrier protein 2 (SDR). (wikipedia.org)
It is a substrate for succinyl-CoA:3-ketoacid-coenzyme A transferase, hydroxymethylglutaryl-CoA synthase, short-chain 3-hydroxyacyl-CoA dehydrogenase, peroxisomal bifunctional enzyme, acetyl-CoA acetyltransferase, and 3-ketoacyl-CoA thiolase. (hmdb.ca)
Peroxisomal bifunctional protein from rat liver is a trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. (rhea-db.org)
This means that the bifunctional protein of rat liver is in fact a trifunctional enzyme possessing delta 3, delta 2-enoyl-CoA isomerase, 2-enoyl-CoA hydratase (EC 4.2.1.17), and L-3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35) activities in the same polypeptide. (rhea-db.org)
We found that the tricarboxylic acid cycle-associated enzyme α-ketoglutarate (α-KG) dehydrogenase (KGDH) entered the nucleus, where it interacted with various JMJs to regulate α-KG-dependent histone demethylations by JMJs, and thus controlled genome-wide gene expression in plants. (bvsalud.org)
Single peroxisomal1
Connect with other caregivers and patients with Disorders with deficiency of a single peroxisomal enzyme and get the support you need. (rareguru.com)
Peroxisome1
The initial description of the peroxisome (originally termed the microbody) appeared in 1954 in a doctoral thesis about mouse kidneys, almost 10 years after the first case description of peroxisomal disease was published. (medscape.com)
Disorders6
Peroxisomal disorders are a group of genetically heterogeneous metabolic diseases that share dysfunction of peroxisomes. (medscape.com)
In peroxisomal biogenesis disorders, abnormal accumulation of VLCFAs (C24, C26) is the hallmark of peroxisomal disorders. (medscape.com)
Peroxisomal disorders are usually caused by a combination of peroxisomal assembly defects or by deficiencies of specific peroxisomal enzymes. (wikipedia.org)
Migrational abnormalities are the most likely causes of the severe seizures and psychomotor retardation associated with many types of peroxisomal disorders. (medscape.com)
Therefore, peroxisomal disorders generally manifest with elevated VLCFA levels (except rhizomelic chondrodysplasia and Refsum disease). (msdmanuals.com)
Biogenesis1
This defect of peroxisomal biogenesis is caused by PEX7 gene mutations and characterized by skeletal changes that include midface hypoplasia, strikingly short proximal limbs, frontal bossing, small nares, cataracts, ichthyosis, and profound psychomotor retardation. (msdmanuals.com)
Nomenclature2
From Enzyme Nomenclature, 1992) EC 2.1.3.2. (uchicago.edu)
Zellweger described the first case of a peroxisomal disorder in the 1940s. (medscape.com)
D-BP deficiency is the most severe peroxisomal disorder, often resembling Zellweger syndrome. (wikipedia.org)
Deficiency4
Rapid whole-genome sequencing identifies a homozygous novel variant, His540Arg, in HSD17B4 resulting in D-bifunctional protein deficiency disorder diagnosis. (nih.gov)
D-Bifunctional protein deficiency is an autosomal recessive peroxisomal fatty acid oxidation disorder. (wikipedia.org)
Defects in MFE2 are a cause of D-bifunctional protein deficiency (DBPD) [ MIM:261515 ]. (mitosciences.com)
This disorder is caused by deficiency of the peroxisomal membrane transporter ALDP, which is coded for by the gene ABCD1 . (msdmanuals.com)
Membrane1
A focus on Yocum and collaborator work who have developed an outstanding approach based on the anchoring of biosynthetic enzymes to the peroxisomal membrane. (univ-tours.fr)
Peroxisomes2
Peroxisomes contain many different enzymes, such as catalase, and their main function is to neutralize free radicals and detoxify drugs. (wikipedia.org)
The D-BP protein contains a peroxisomal targeting signal 1 (PTS1) unit at the C-terminus allowing for its transport into peroxisomes by the PTS1 receptor. (wikipedia.org)
Catalyzes3
An enzyme that catalyzes the conversion of carbamoyl phosphate and L-aspartate to yield orthophosphate and N-carbamoyl-L-aspartate. (uchicago.edu)
An enzyme that catalyzes the reversible isomerization of D-mannose-6-phosphate to form D-fructose-6-phosphate, an important step in glycolysis. (lookformedical.com)
Along with animal peroxidases, these enzymes belong to a group of peroxidases containing a heme prosthetic group (ferriprotoporphyrin IX), which catalyzes a multistep oxidative reaction involving hydrogen peroxide as the electron acceptor. (unl.edu)
Proteins2
Candidates for each step are identified by using ublast (a fast alternative to protein BLAST) against a database of manually-curated proteins (most of which are experimentally characterized) or by using HMMer with enzyme models (usually from TIGRFam ). (lbl.gov)
Using a newly developed enzyme-coupled continuous activity assay, we compare differing NAS proteins identified through multiple sequence alignments and phylogenetic analyses. (bvsalud.org)
Gene3
The responsible genetic defect occurs in 1 of at least 12 genes involved in peroxisomal formation or protein import (the PEX gene family). (msdmanuals.com)
Gene sets follow the order of enzymes. (umassmed.edu)
Not available " indicates that no score was available for the corresponding gene, enzyme, or reaction in the relevant database. (umassmed.edu)
Sequence1
Comparison of the amino acid sequences of cyanogen bromide cleaved isomerase with the known sequence of the peroxisomal bifunctional protein from the rat identified them as the same molecule. (rhea-db.org)
Mitochondrial1
The mitochondrial and peroxisomal isoenzymes were separated chromatographically and the peroxisomal isomerase purified to apparent homogeneity. (rhea-db.org)
Isomerization1
In control experiments, the peroxisomal bifunctional protein purified according to published methods also catalyzed delta 3, delta 2-enoyl-CoA isomerization. (rhea-db.org)
Fatty acids1
These enzymes overlap in function with those in mitochondria, with the exception that mitochondria lack enzymes to metabolize very long-chain fatty acids (VLCFA), those 20 to 26 carbons in length. (msdmanuals.com)
Liver2
Peroxisomal delta 3, delta 2-enoyl-CoA isomerase (EC 5.3.3.8) was studied in the liver of rats treated with clofibrate. (rhea-db.org)
In immunoblotting it was recognized by the antibody to peroxisomal bifunctional protein from rat liver. (rhea-db.org)
Distinct2
They are distinguished from MULTIENZYME COMPLEXES in that their subunits are not found as distinct enzymes. (rush.edu)
Through this commentary article, we highlight the outstanding work of the Zhou's group which recently describes a distinct approach relying on the efficient recycling of enzyme co-factors in yeast to fulfill the synthesis of caffeic acid (CaA) and ferulic acid (FA), two precursors of podophyllotoxin. (univ-tours.fr)
Synthesis1
We propose that the specificity of XXT3, XXT4, and XXT5 is directed toward the prior synthesis of the acceptor substrate by the other two enzymes, XXT1 and XXT2. (bvsalud.org)
Specific2
Reaction for 5.3.3.8 is not in the list of KEGG reactions as specific matches are not available for this enzyme in the KEGG database. (umassmed.edu)
specific protein associated with the reaction, generic enzyme associated with the reaction, and the reaction itself. (umassmed.edu)
Group2
A group of enzymes that catalyze an intramolecular transfer of a phosphate group. (lookformedical.com)
It has been shown in some cases that the enzyme has a functional phosphate group, which can act as the donor. (lookformedical.com)
Publications2
This graph shows the total number of publications written about "Multifunctional Enzymes" by people in this website by year, and whether "Multifunctional Enzymes" was a major or minor topic of these publications. (rush.edu)
Below are the most recent publications written about "Multifunctional Enzymes" by people in Profiles. (rush.edu)
Description6
Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Human Angiomotin (AMOT) in Tissue homogenates, cell lysates and other biological fluids. (myelisakit.com)
Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Human Angiomotin (AMOT) in samples from Tissue homogenates, cell lysates and other biological fluids with no significant corss-reactivity with analogues from other species. (myelisakit.com)
Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Mouse Hepatocyte Nuclear Factor 1 Alpha (HNF1a) in Tissue homogenates, cell lysates and other biological fluids. (wildpalm.net)
Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Mouse Hepatocyte Nuclear Factor 1 Alpha (HNF1a) in samples from Tissue homogenates, cell lysates and other biological fluids with no significant corss-reactivity with analogues from other species. (wildpalm.net)
Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Human GA Binding Protein Transcription Factor Alpha (GABPa) in serum, plasma, tissue homogenates, cell lysates and other biological fluids. (therabio.org)
Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Human GA Binding Protein Transcription Factor Alpha (GABPa) in samples from serum, plasma, tissue homogenates, cell lysates and other biological fluids with no significant corss-reactivity with analogues from other species. (therabio.org)