磷酸羧化酶2重组兔单抗

Rrmab®兔单抗
2026-01-04~2026-02-28,RR26012026-01-04~2026-02-28,TR
磷酸羧化酶2重组兔单抗
货号:bsm-60457R
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概述

产品编号
bsm-60457R
产品类型
重组兔单抗
英文名称
PCK2 Recombinant Rabbit mAb
中文名称
磷酸羧化酶2重组兔单抗
英文别名
PCKGM_HUMAN; PCK2; PEPCK-M; Phosphoenolpyruvate carboxykinase 2, mitochondrial (mtPCK2); 4.1.1.32; PEPCK2; PEPCK; mtPCK2;
抗体来源
Rabbit
亚型
IgG/Kappa
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Recombinant
克隆号
E6C11
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
背景资料
PCK2 is a a member of the phosphoenolpyruvate carboxykinase (GTP) family. The protein is a mitochondrial enzyme that catalyzes the conversion of oxaloacetate to phosphoenolpyruvate in the presence of GTP. A cytosolic form encoded by a different gene has also been characterized and is the key enzyme of gluconeogenesis in the liver. The encoded protein may serve a similar function, although it is constitutively expressed and not modulated by hormones such as glucagon and insulin that regulate the cytosolic form. Alternatively spliced transcript variants have been described.
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产品应用

应用已检合格种属预测种属推荐稀释比例
WBHuman, Mouse, Rat1:500-2000
IHC-PHuman, Mouse, Rat1:100-500
IHC-FHuman, Mouse, Rat1:100-500
IFHuman, Mouse, Rat1:100-500
Flow-CytHumanMouse, Rat1:50-100

交叉反应

交叉反应: Human, Mouse, Rat

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靶标

基因名
PCK2
蛋白名
phosphoenolpyruvate carboxykinase [GTP], mitochondrial
亚基
Monomer.
亚细胞定位
Mitochondrion.
翻译后修饰
Phosphorylated upon DNA damage, probably by ATM or ATR.
疾病
Defects in PCK2 are the cause of mitochondrial phosphoenolpyruvate carboxykinase deficiency (M-PEPCKD) [MIM:261650]. A metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait.
相似性
Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
功能
Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle (By similarity).

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