antibody

Goat anti-AMACR (aa312-326) Antibody

MBS423435

0.1 mg

305 USD

polyclonal

goat

nonconjugated

western blot, enzyme immunoassay

Antibody

Goat anti-AMACR (aa312-326) Antibody

[AMACR]

[AMACR, alpha-methylacyl-CoA racemase, AMACRD, CBAS4, RACE, RM, 2-methylacyl-CoA racemase]

[alpha-methylacyl-CoA racemase isoform 1; Alpha-methylacyl-CoA racemase; alpha-methylacyl-CoA racemase; alpha-methylacyl-CoA racemase; 2-methylacyl-CoA racemase]

[AMACR]

[AMACR; AMACR; RM; RACE; CBAS4; AMACRD]

AMACR_HUMAN

Polyclonal

Goat

Tested: Human; Expected from sequence similarity: Human

382

RGSFITSEEQDVSPR

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Peptide ELISA (EIA), Western Blot (WB)

Peptide ELISA: Antibody detection limit dilution 1: 64000. Western Blot: Approx 45kDa band observed in Human Kidney and Liver lysates (calculated MW of 42.4kDa according to NP_055139.4). Recommended concentration: 0.1-0.3ug/ml.

Western Blot (WB)

Immunogen: Peptide with sequence C-RGSFITSEEQDVSPR, from the internal region of the protein sequence according to NP_055139.4; NP_001161067.1.

Note: This antibody is expected to recognize isoform 1 (NP_055139.4) and isoform 3 (NP_001161067.1).

42794625

NP_055139.4

NM_014324.5

43,860 Da

Beta-oxidation Of Pristanoyl-CoA Pathway (1270033); Bile Acid And Bile Salt Metabolism Pathway (1270040); Bile Acid Biosynthesis, Cholesterol = Cholate/chenodeoxycholate Pathway (413432); Bile Acid Biosynthesis, Cholesterol = Cholate/chenodeoxycholate Pathway (468297); Metabolic Pathways (132956); Metabolism Pathway (1269956); Metabolism Of Lipids And Lipoproteins Pathway (1270001); Peroxisomal Lipid Metabolism Pathway (1270031); Peroxisome Pathway (131226); Peroxisome Pathway (131126)

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]

AMACR: 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. Belongs to the CaiB/BaiF CoA-transferase family. 3 isoforms of the human protein are produced by alternative splicing. Protein type: Lipid Metabolism - primary bile acid biosynthesis; Isomerase; Mitochondrial; EC 5.1.99.4. Chromosomal Location of Human Ortholog: 5p13. Cellular Component: cytoplasm; mitochondrion; peroxisomal matrix; peroxisome. Molecular Function: alpha-methylacyl-CoA racemase activity; receptor binding. Biological Process: bile acid biosynthetic process; bile acid metabolic process; fatty acid beta-oxidation using acyl-CoA oxidase. Disease: Alpha-methylacyl-coa Racemase Deficiency; Bile Acid Synthesis Defect, Congenital, 4

0.1 mg

305 USD