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产品信息
PA1-23482
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Rabbit Polyclonal to Q(399) D K I F Q G N K D Y H K D V R N N(416) of the human ESDN Protein
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Affinity chromatography
保存液
PBS
内含物
0.02% sodium azide
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
Ambient (domestic); Wet ice (international)
RRID
AB_2277084
产品详细信息
PA1-23482 detects ESDN in Human samples.
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 fpropionic acidemia type I (PA-1) [MIM:606054]. 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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生物信息学
蛋白别名: 1700055P21Rik; coagulation factor V/VIII-homology domains protein 1; CUB, LCCL and coagulation factor V/VIII-homology domains protein 1; discoidin; Discoidin, CUB and LCCL domain-containing protein 2; Endothelial and smooth muscle cell-derived neuropilin-like protein
基因别名: CLCP1; DCBLD2; ESDN
UniProt ID: (Human) Q96PD2
Entrez Gene ID: (Human) 131566
