AMACR
This gene encodes a racemase. The encoded enzyme interconverts pristanoyl-CoA and C27-bile acylCoAs between their (R)- and (S)-stereoisomers. The conversion to the (S)-stereoisomers is necessary for degradation of these substrates by peroxisomal beta-oxidation. Encoded proteins from this locus localize to both mitochondria and peroxisomes. Mutations in this gene may be associated with adult-onset sensorimotor neuropathy, pigmentary retinopathy, and adrenomyeloneuropathy due to defects in bile acid synthesis. Alternatively spliced transcript variants have been described. Read-through transcription also exists between this gene and the upstream neighboring C1QTNF3 (C1q and tumor necrosis factor related protein 3) gene.
Full Name
Alpha-Methylacyl-CoA Racemase
Alternative Names
Alpha-Methylacyl-CoA Racemase; 2-Methylacyl-CoA Racemase; EC 5.1.99.4; AMACRD; CBAS4; P504S; RACE; RM;
Function
Catalyzes the interconversion of (R)- and (S)-stereoisomers of alpha-methyl-branched-chain fatty acyl-CoA esters (PubMed:7649182, PubMed:10655068, PubMed:11060359). Acts only on coenzyme A thioesters, not on free fatty acids, and accepts as substrates a wide range of alpha-methylacyl-CoAs, including pristanoyl-CoA, trihydroxycoprostanoyl-CoA (an intermediate in bile acid synthesis), and arylpropionic acids like the anti-inflammatory drug ibuprofen (2-(4-isobutylphenyl)propionic acid) but neither 3-methyl-branched nor linear-chain acyl-CoAs (PubMed:7649182, PubMed:10655068, PubMed:11060359).
Biological Process
Bile acid biosynthetic process Source: Reactome
Bile acid metabolic process Source: UniProtKB
Fatty acid beta-oxidation using acyl-CoA oxidase Source: Reactome
Protein localization Source: Reactome
Bile acid metabolic process Source: UniProtKB
Fatty acid beta-oxidation using acyl-CoA oxidase Source: Reactome
Protein localization Source: Reactome
Cellular Location
Peroxisome; Mitochondrion
Involvement in disease
Alpha-methylacyl-CoA racemase deficiency (AMACRD): A rare autosomal recessive peroxisomal disorder characterized by elevated plasma concentrations of pristanic acid C27-bile-acid intermediates, and adult onset of variable neurodegenerative symptoms affecting the central and peripheral nervous systems. Features may include seizures, visual failure, sensorimotor neuropathy, spasticity, migraine, and white matter hyperintensities on brain imaging.
Congenital bile acid synthesis defect 4 (CBAS4): A disorder characterized by the presence of trihydroxycoprostanic acid in the bile and absence of cholic acid. Patients manifest neonatal jaundice, intrahepatic cholestasis and bile duct deficiency.
Congenital bile acid synthesis defect 4 (CBAS4): A disorder characterized by the presence of trihydroxycoprostanic acid in the bile and absence of cholic acid. Patients manifest neonatal jaundice, intrahepatic cholestasis and bile duct deficiency.
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Anti-AMACR antibodies
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CompareTarget: AMACR
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human, Mouse, Rat
Clone: CB34A
Application*: WB, IP, IF, E
Target: AMACR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CB012
Application*: IHC
Target: AMACR
Host: Mouse
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat
Clone: EC13
Application*: WB, P, IF
Target: AMACR
Host: Mouse
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat
Clone: CBT6
Application*: WB, IF, IC, P
Target: AMACR
Host: Mouse
Antibody Isotype: IgG
Specificity: Human, Mouse
Clone: CBT152
Application*: WB, P, IF, IC, E
Target: AMACR
Host: Mouse
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat
Clone: CBT975
Application*: WB, IF, IC
Target: AMACR
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Mouse
Clone: CBT3651
Application*: WB, IH, IC
Target: AMACR
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human, Mouse, Rat
Clone: CB128A
Application*: WB, IP, IF, E
Target: AMACR
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human, Mouse, Rat
Clone: CB86A
Application*: WB, IP, IF, E
Target: AMACR
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: RM349
Application*: IHC, WB
Target: AMACR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse
Clone: 2H9B5
Application*: FC, WB
Target: AMACR
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Mouse, Rat
Clone: 2A10F3
Application*: WB, P, IC, IF, IP
Target: AMACR
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: 1D8
Application*: E, WB
Target: AMACR
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat
Clone: CBFYR-0002
Application*: WB, IH
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(P): Predicted
* Abbreviations
- AActivation
- AGAgonist
- APApoptosis
- BBlocking
- BABioassay
- BIBioimaging
- CImmunohistochemistry-Frozen Sections
- CIChromatin Immunoprecipitation
- CTCytotoxicity
- CSCostimulation
- DDepletion
- DBDot Blot
- EELISA
- ECELISA(Cap)
- EDELISA(Det)
- ESELISpot
- EMElectron Microscopy
- FFlow Cytometry
- FNFunction Assay
- GSGel Supershift
- IInhibition
- IAEnzyme Immunoassay
- ICImmunocytochemistry
- IDImmunodiffusion
- IEImmunoelectrophoresis
- IFImmunofluorescence
- IHImmunohistochemistry
- IMImmunomicroscopy
- IOImmunoassay
- IPImmunoprecipitation
- ISIntracellular Staining for Flow Cytometry
- LALuminex Assay
- LFLateral Flow Immunoassay
- MMicroarray
- MCMass Cytometry/CyTOF
- MDMeDIP
- MSElectrophoretic Mobility Shift Assay
- NNeutralization
- PImmunohistologyp-Paraffin Sections
- PAPeptide Array
- PEPeptide ELISA
- PLProximity Ligation Assay
- RRadioimmunoassay
- SStimulation
- SESandwich ELISA
- SHIn situ hybridization
- TCTissue Culture
- WBWestern Blot
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