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Rabbit Anti-HLCS/AP Conjugated antibody (bs-18044R-AP)
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訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-18044R-AP
英文名稱1 Rabbit Anti-HLCS/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標記的生物素蛋白連接酶抗體
別    名 Biotin [acetyl CoA carboxylase] ligase; Biotin [methylcrotonoyl CoA carboxylase] ligase; Biotin [methylmalonyl CoA carboxytransferase] ligase; Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase; Biotin apo protein ligase; Biotin apo-protein ligase; Biotin protein ligase; Biotin--[acetyl-CoA-carboxylase] ligase; BPL1_HUMAN; HCS; HLCS; Holocarboxylase synthetase; Holocarboxylase synthetase, EC 6.3.4.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細胞生物  免疫學  信號轉(zhuǎn)導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Dog, )
產(chǎn)品應(yīng)用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 80kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HLCS
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:
This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene are the cause of holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jun 2011]

Function:
Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase.

Subcellular Location:
Cytoplasm. Mitochondrion.

Tissue Specificity:
Mostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung.

DISEASE:
Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency) [MIM:253270]; also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin.

Similarity:
Belongs to the biotin--protein ligase family.

Database links:

Entrez Gene: 3141 Human

Entrez Gene: 110948 Mouse

Entrez Gene: 288240 Rat

Omim: 609018 Human

SwissProt: P50747 Human

SwissProt: Q920N2 Mouse

Unigene: 371350 Human

Unigene: 30921 Mouse



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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