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货号： bs-21070R-PE-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-21070R-PE-Cy3
- 英文名称
- Anti-P5CS/PE-Cy3
- 中文名称
- PE-Cy3标记的γ-5羧酸合成酶抗体
- 别 名
- 2810433K04Rik; AI429789; Aldehyde dehydrogenase 18 family member A1; Aldehyde dehydrogenase 18A1; Aldehyde dehydrogenase family 18 member A1; ALDH18A1; Delta 1 pyrroline 5 carboxylate synthetase; Delta1 pyrroline 5 carboxlate synthetase; Gamma-glutamyl kinase; Gamma-glutamyl phosphate reductase; GK; Glutamate-5-semialdehyde dehydrogenase; Glutamyl-gamma-semialdehyde dehydrogenase; GPR; GSAS; MGC117316; MGC32233; P5CS; P5CS_HUMAN; PYCS; Pyrroline 5 carboxylate synthetase (glutamate gamma semialdehyde synthetase).
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Dog, Pig, Cow, Horse, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 87kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human P5CS
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- 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.
- 产品介绍
- background:
This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene. [provided by RefSeq, Jul 2008]
Function:
Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, ornithine and arginine.
Subcellular Location:
Mitochondrion inner membrane.
DISEASE:
Defects in ALDH18A1 are the cause of mental retardation-joint hypermobility-skin laxity with or without metabolic abnormalities (MRJHSL) [MIM:612652]. Clinical manifestations include microcephaly, progressive neurologic dysfunction, mental retardation, progeroid appearance, joint hypermobility, skin laxity and hyperelasticity, cataracts. Some patients manifest metabolic disturbances such as hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia.
Similarity:
In the N-terminal section; belongs to the glutamate 5-kinase family.
In the C-terminal section; belongs to the gamma-glutamyl phosphate reductase family.
Database links:Entrez Gene: 514759Cow
Entrez Gene: 477781Dog
Entrez Gene: 100061213Horse
Entrez Gene: 5832Human
Entrez Gene: 56454Mouse
Entrez Gene: 100151977Pig
Entrez Gene: 361755Rat
Omim: 138250Human
SwissProt: P54886Human
SwissProt: Q9Z110Mouse
Unigene: 500645Human
Unigene: 233117Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.