AMACR (clone 2A10F3), anti-human, mouse
€476.00
In stock
SKU
252728
Catalog Nr.: 252728
Size: 0.1 ml
Isotype: Mouse IgG2b
Applications: WB, IHC, IF
Size: 0.1 ml
Isotype: Mouse IgG2b
Applications: WB, IHC, IF
Protein Family: Enzymes
Pathway and Disease: Lipid Metabolism
Description:
AMACR (Alpha-methylacyl-CoA racemase) catalyzes the racemization of 2-methyl-branched fatty acid CoA esters. AMACR is responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers. Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD). AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. AMACRD is associated with polyneuropathy, retinitis pigmentosa and epilepsy.
Alternate Names: AMACR, Alpha-methylacyl-CoA racemase, 2-methylacyl-CoA racemase, RACE
Application Notes: WB: 1:500-1:2000; IHC: 1:200-1:1000; IF: 1:200-1:1000
Accession No.: Q9UHK6
Antigen: Purified recombinant fragment of human AMACR expressed in E. coli.
Format: Each vial contains 0.1 ml ascitic fluid with 0.03% sodium azide.
Storage:
Store at 4°C for short term use only. Store at -20°C for storage over 1 month. Product is guaranteed 6 months from the date of shipment.
Pathway and Disease: Lipid Metabolism
Description:
AMACR (Alpha-methylacyl-CoA racemase) catalyzes the racemization of 2-methyl-branched fatty acid CoA esters. AMACR is responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers. Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD). AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. AMACRD is associated with polyneuropathy, retinitis pigmentosa and epilepsy.
Alternate Names: AMACR, Alpha-methylacyl-CoA racemase, 2-methylacyl-CoA racemase, RACE
Application Notes: WB: 1:500-1:2000; IHC: 1:200-1:1000; IF: 1:200-1:1000
Accession No.: Q9UHK6
Antigen: Purified recombinant fragment of human AMACR expressed in E. coli.
Format: Each vial contains 0.1 ml ascitic fluid with 0.03% sodium azide.
Storage:
Store at 4°C for short term use only. Store at -20°C for storage over 1 month. Product is guaranteed 6 months from the date of shipment.
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