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    Rabbit Anti-PCK2/BF488 Conjugated antibody (bs-5002R-BF488)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-5002R-BF488
    英文名稱(chēng) Rabbit Anti-PCK2/BF488 Conjugated antibody
    中文名稱(chēng) BF488標記的磷酸羧化酶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.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域 腫瘤  心血管  細胞生物  免疫學(xué)  信號轉導  激酶和磷酸酶  脂蛋白  線(xiàn)粒體  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, )
    產(chǎn)品應用 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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