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货号： bs-9808R-PE-Cy5 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9808R-PE-Cy5
- 英文名称
- Anti-C2ORF25/PE-Cy5
- 中文名称
- PE-Cy5标记的2号染色体开放阅读框25抗体
- 别 名
- cblD; Chromosome 2 open reading frame 25; Protein C2orf25, mitochondrial; CL25022; Methylmalonic aciduria (cobalamin deficiency) cblD type, with homocystinuria; Protein C2orf25, mitochondrial; MMAD_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 29kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human C2ORF25
- 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:
C2ORF25 is a mitochondrial protein and its function is not fully identified. But, Vitamin B12 (cobalamin) is an essential cofactor in several metabolic pathways. Intracellular conversion of cobalamin to adenosylcobalamin in mitochondria and to methylcobalamin in cytoplasm is necessary for homeostasis of methylmalonic acid and homocysteine. C2ORF25 encodes a protein involved in an early step of cobalamin metabolism.
Function:
nvolved in cobalamin metabolism.
Subcellular Location:
Mitochondrial.
Tissue Specificity:
Widely expressed at high levels.
DISEASE:
Defects in MMADHC are the cause of methylmalonic aciduria and homocystinuria type cblD (MMAHCD) [MIM:277410]. A disorder of cobalamin metabolism characterized by decreased levels of the coenzymes adenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl). Clinical features include developmental delay, hyotonia, mental retardation, seizures, megaloblastic anemia. Some patients manifest combined methylmalonic aciduria and homocystinuria (referred to as cblD original), some have only isolated homocystinuria (cblD variant 1), and others have only methylmalonic aciduria (cblD variant 2).
Database links:Entrez Gene: 27249 Human
Entrez Gene: 109129 Mouse
Entrez Gene: 362134 Rat
Omim: 611935 Human
SwissProt: Q9H3L0 Human
SwissProt: Q99LS1 Mouse
SwissProt: Q6AYQ6 Rat
Unigene: 5324 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.