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Anti-AMACR Antibody

品牌：Antibodies

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規(guī)格：100μg

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Anti-AMACR Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

Overview

Name:	Anti-AMACR Antibody See all AMACR primary antibodies
Description:	Goat polyclonal antibody to AMACR.
Applications:	ELISA, WB
Reactivity:	Human
Immunogen:	Synthetic peptide corresponding to Human AMACR (C terminal).
Sequence:	C-QLNSDKIIESN
Host:	Goat
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration:	100 μg at 0.5 mg/ml.
Product Form:	Liquid
Formulation:	Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Target

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.
Involvement in Disease:	Alpha-methylacyl-CoA racemase deficiency: A rare autosomal recessive peroxisomal disorder characterized by elevated plasma concentrations of pristanic acid C27-bile-acid intermediates, and ***** 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: 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.
Sequence Similarities:	Belongs to the CaiB/BaiF CoA-transferase family.
Cellular Location:	Peroxisome. Mitochondrion.
Database Links:	Entrez Gene: 23600?Human Omim: 604489?Human SwissProt: Q9UHK6?Human Unigene: 508343?Human
Synonyms:	2 arylpropionyl CoA epimerase Antibody 2 methylacyl CoA racemase Antibody 2-methylacyl-CoA racemase Antibody Alpha methylacyl CoA racemase Antibody Alpha methylacyl Coenzyme A racemase Antibody Alpha methylacyl-CoA racemase deficiency, included Antibody Alpha-methylacyl-CoA racemase Antibody AMACR Antibody AMACR deficiency, included Antibody AMACR_HUMAN Antibody CBAS4 Antibody Da1-8 Antibody EC 5.1.99.4 Antibody Macr1 Antibody Methylacyl CoA racemase alpha Antibody RACE Antibody RM Antibody
Information:	Target information shown above is from the UniProt Consortium.