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PCK2 Rabbit pAb (bs-5002R)  
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產品編號 bs-5002R
英文名稱 PCK2 Rabbit pAb
中文名稱 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; phosphoen  
Specific References  (2)     |     bs-5002R has been referenced in 2 publications.
[IF=4.533] Huiying Gu. et al. Soluble Klotho Improves Hepatic Glucose and Lipid Homeostasis in Type 2 Diabetes. Mol Ther-Meth Clin D. 2020 Sep;18:811  WB ;  Mouse.  
[IF=2.718] Naomi Nishio. et al. Hen egg only diets support healthy aging in adult mice. J ANIM PHYSIOL AN N. 2023 Jan;:  WB ;  Mouse.  
研究領域 腫瘤  心血管  細胞生物  免疫學  信號轉導  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 (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Horse)
產品應用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 67 kDa
檢測分子量
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PCK2: 365-470/640 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
Q16822

Gene ID:
5106

Database links:
UniProtKB/Swiss-Prot: Q16822.3

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