磷酸羧化酶2抗体
规格：1mg/1ml
英文名： PCK2
别名： EC 4.1.1.32; GTP mitochondrial precursor; HGNC:8725; mitochondrial; Mitochondrial phosphoenolpyruvate carboxykinase 2; OTTHUMP00000164700; PCK2; PCKGM_HUMAN; PE; PEP carboxykinase; PEPCK; PEPCK defici
分子量： 67kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PCK2
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Rabbit,
细胞定位：细胞浆 线粒体
磷酸羧化酶2抗体产品介绍：background: 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. Function: 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磷酸羧化酶2抗体 the citric acid cycle (By similarity). Subunit: Monomer. Subcellular Location: Mitochondrion. Post-translational modifications: Phosphorylated upon DNA damage, probably by ATM or ATR. DISEASE: 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 磷酸羧化酶2抗体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. Similarity: Belongs to the phosphoenolpyruvate carboxykinase [GTP] family. Database links: UniProtKB/Swiss-Prot: Q16822.3 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
磷酸羧化酶2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 心血管 细胞生物 **学 信号转导 激酶和磷酸酶 脂蛋白 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid