Name: AMACR [C-term] mouse mAb
SKU: WM0615
Price: 268 USD
Availability: InStock

AMACR [C-term] mouse mAb

Ucallm_WM0615-50

Price range: $268.00 through $328.00

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50μl

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SKU: WM0615-50

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Product details

Background:
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. [provided by RefSeq, Mar 2011],

Specifications

Size	50μL/100μL
Target	AMACR
Reactivity	Rat
Applications	WB, ICC
MW(Calculated)
MW(Observed)	42kD
Host Species	Mouse
Isotype
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:1000; ICC 1:100
Formulation	Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Purification	The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	4B7
Immunogen	Purified recombinant human AMACR(C-terminus) protein fragments expressed in E.coli.
Specificity	This antibody detects endogenous levels of AMACR(C-terminus) and does not cross-react with related proteins.
Gene Name	amacr
Protein Name
Other Name	2 arylpropionyl CoA epimerase; / 2 methylacyl CoA racemase; / 2-methylacyl-CoA racemase; / Alpha methylacyl CoA racemase; / Alpha methylacyl Coenzyme A racemase; / Alpha methylacyl-CoA racemase deficiency, included; / Alpha-methylacyl-CoA racemase; / Amacr; / AMACR deficiency, included; / AMACR_HUMAN; / CBAS4; / Da1-8; / Macr 1; / Macr1; / Methylacyl CoA racemase alpha; / P504S; / RACE; / RM.
Species	Human
Gene ID	23600
Uniprot	Q9UHK6,
Species.1	Mouse
Gene ID.1
Uniprot.1	O09174
Species.2
Gene ID.2
Uniprot.2
Cellular Localization	Peroxisome . Mitochondrion .
Tissue Expression	Aorta,Brain,Cerebellum,Kidney,Liver,PCR rescued clones,Prostate cancer,Sali
Signaling_pathway	Cellular Processes >> Transport and catabolism >> Peroxisome
Research Areas	>>Primary bile acid biosynthesis; / >>Metabolic pathways; / >>Peroxisome
Function	Catalytic activity:(2S)-2-methylacyl-CoA = (2R)-2-methylacyl-CoA.,Disease:Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:604489]. AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy.,Disease:Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) [MIM:214950]; also known as cholestasis, intrahepatic, with defective conversion of trihydroxycoprostanic acid to cholic acid or trihydroxycoprostanic acid in bile. Clinical features include neonatal jaundice, intrahepatic cholestasis, bile duct deficiency and absence of cholic acid from bile.,Function:Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers.,pathway:Lipid metabolism; bile acid biosynthesis.,pathway:Lipid metabolism; fatty acid metabolism.,similarity:Belongs to the caiB/baiF CoA-transferase family.,similarity:Contains 1 C1q domain.,similarity:Contains 1 collagen-like domain.,