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Rabbit Anti-PCK2/BF350 Conjugated antibody (bs-5002R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-5002R-BF350
英文名稱1 Rabbit Anti-PCK2/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的磷酸羧化酶2抗體
別    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 deficiency mitochondrial; PEPCK M; PEPCK-M; PEPCK2; Phosphoenolpyruvate carboxykinase [GTP]; Phosphoenolpyruvate carboxykinase 2 (mitochondrial); Phosphoenolpyruvate carboxykinase 2 mitochondrial; Phosphoenolpyruvate carboxylase; Phosphopyruvate carboxylase.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  心血管  細(xì)胞生物  免疫學(xué)  信號轉(zhuǎn)導(dǎo)  激酶和磷酸酶  脂蛋白  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 67kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PCK2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 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 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 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.
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