5.1.99.4: alpha-methylacyl-CoA racemase
This is an abbreviated version!
For detailed information about alpha-methylacyl-CoA racemase, go to the full flat file.
Word Map on EC 5.1.99.4
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5.1.99.4
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prostate
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adenocarcinoma
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papillary
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cytokeratins
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high-grade
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gleason
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prostatectomy
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neoplasia
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needle
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clinicopathologic
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vimentin
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intraepithelial
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prostate-specific
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eosinophilic
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urothelial
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acinar
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pathologist
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immunophenotype
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34betae12
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hgpin
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chromophobe
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atypia
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pristanic
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oncocytic
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suspicious
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oncocytomas
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transurethral
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nucleoli
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drug development
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tmprss2-erg
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metanephric
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fuhrman
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immunomarkers
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chrcc
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cribriform
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analysis
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nephrogenic
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medicine
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psammoma
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pharmacology
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sarcomatoid
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hobnail
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tubulocystic
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immunoprofile
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adenosis
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phytanic
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diagnostics
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tubulopapillary
- 5.1.99.4
- prostate
- adenocarcinoma
-
papillary
-
cytokeratins
-
high-grade
-
gleason
-
prostatectomy
- neoplasia
-
needle
-
clinicopathologic
- vimentin
-
intraepithelial
-
prostate-specific
-
eosinophilic
-
urothelial
-
acinar
-
pathologist
-
immunophenotype
-
34betae12
-
hgpin
-
chromophobe
-
atypia
-
pristanic
-
oncocytic
-
suspicious
- oncocytomas
-
transurethral
-
nucleoli
- drug development
-
tmprss2-erg
-
metanephric
-
fuhrman
-
immunomarkers
-
chrcc
-
cribriform
- analysis
-
nephrogenic
- medicine
-
psammoma
- pharmacology
-
sarcomatoid
-
hobnail
-
tubulocystic
-
immunoprofile
-
adenosis
-
phytanic
- diagnostics
-
tubulopapillary
Reaction
Synonyms
2-arylpropionyl-CoA epimerase, 2-methylacyl-CoA racemase, alpha-methyl CoA racemase, alpha-Methylacyl CoA racemase, alpha-methylacyl coenzyme A racemase, alpha-methylacyl-CoA racemase, alpha-methylacyl-CoA racemase 1A, alpha-methylacyl-coenzyme A racemase, AMACR, AMACR 1A, AMACR IA, amacr/P504S, GenBank U89905-derived protein GI2145184, GenBank U89906-derived protein GI 2145186, ibuprofenoyl-CoA epimerase, MCR, P504S, Racemase, alpha-methylacyl coenzyme A, Racemase, alpha-methylacyl coenzyme A (Mus musculus clone 3), Racemase, alpha-methylacyl coenzyme A (Rattus norvegicus clone 11)
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General Information
General Information on EC 5.1.99.4 - alpha-methylacyl-CoA racemase
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malfunction
metabolism
physiological function
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alpha-methylacyl coenzyme A racemase is positive in most cancers and dysplasias but only rarely in nonneoplastic epithelium
malfunction
overexpression of alpha-methylacyl-CoA racemase is associated with CTNNB1 mutations in hepatocellular carcinomas
beta-oxidation of branched-chain fatty acids, important enzyme for the metabolism of branched-chain lipids and drugs
alpha-methylacyl-CoA racemase catalyses the inversion of configuration at the 2-position of fatty acyl-CoA esters from 2R to 2S and controls entry of metabolites into peroxisomal beta-oxidation
physiological function
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alpha-methylacyl-CoA racemase is a protein that plays an important role in mitochondrial and peroxisomal beta-oxidation of branched-chain fatty acid and bile acid intermediates
physiological function
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enzyme catalyzes the 1,1-proton transfer reaction that effects the reversible epimerization of the coenzyme A thioesters derived from a variety of (2R)- and (2S)-methyl branched fatty acids
physiological function
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enzyme catalyzes the racemization of alpha-methyl carboxylic coenzyme A thioesters in mitochondria and peroxisomes, induction of AMACR-specific cytotoxic mononuclear cells from prostate cancer patients peripheral blood mononuclear cells
physiological function
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enzyme is involved in the beta-oxidation of branched-chain fatty acids
physiological function
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AMACR is involved in the beta-oxidation of the dietary branched-chain fatty acids and bile acid intermediate
physiological function
AMACR is involved in the beta-oxidation of the dietary branched-chain fatty acids and bile acid intermediate
physiological function
the enzyme plays a gate-keeping role in the beta-oxidation of branched-chain fatty acid CoA esters, e.g. pristanoyl-CoA, catalyzing the racemization of the 2-R-configured CoA ester to the S-configured stereoisomer just prior to beta-oxidation. This is an obligatory step, as the first stage of beta-oxidation is catalyzed by an oxidase that accepts only S-configured 2-methylalkanoic CoA esters
physiological function
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the enzyme plays an important role in the beta-oxidation of branched-chain fatty acid and its derivatives
physiological function
knockout mouse models deficient in AMACR or MFE-2 (peroxisomal multifunctional enzyme type 2), in which this beta-oxidation pathway is prevented, display a residual C24-bile acid pool which, although greatly reduced, implies the existence of alternative pathways of bile acid synthesis. The total bile acid pool is decreased in Mfe-1-/- mice compared with wild-type and the levels of mature C24-bile acids are reduced in the double knockout mice when compared with AMACR-deficient mice
physiological function
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knockout mouse models deficient in AMACR or MFE-2 (peroxisomal multifunctional enzyme type 2), in which this beta-oxidation pathway is prevented, display a residual C24-bile acid pool which, although greatly reduced, implies the existence of alternative pathways of bile acid synthesis. The total bile acid pool is decreased in Mfe-1-/- mice compared with wild-type and the levels of mature C24-bile acids are reduced in the double knockout mice when compared with AMACR-deficient mice
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