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Invitrogen
PCCA Polyclonal Antibody
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产品信息
PA1-27572
产品规格
宿主/亚型
Chicken
/ IgY
分类
Polyclonal
类型
Antibody
抗原
Synthetic peptide corresponding to human Propionyl-CoA carboxylase alpha chain. Mitochondrial.
偶联物
形式
Liquid
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Wet ice
RRID
产品详细信息
By Western blot, this antibody has only been tested on fusion protein.
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
靶标信息
This protein is a key enzyme in the catabolic pathway of odd-chain fatty acids, isoleucine, threonine, methionine, and valine. It is probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. The protein is located in the mitochondrial matrix. Defects in PCCA are the cause o propionic acidemia type I (PA-1). PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, develop-mental retardation, and intolerance to protein.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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