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Rabbit Anti-C2ORF25/Gold Conjugated antibody (bs-9808R-Gold)
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-9808R-Gold
英文名稱1 Rabbit Anti-C2ORF25/Gold Conjugated antibody
中文名稱 膠體金標記的2號染色體開放閱讀框25抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 細胞生物  免疫學  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, )
產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 29kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human C2ORF25
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 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.
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