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Rabbit Anti-SLC25A19/Gold Conjugated antibody (bs-21222R-Gold)
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-21222R-Gold
英文名稱1 Rabbit Anti-SLC25A19/Gold Conjugated antibody
中文名稱 膠體金標記的溶質載體家族25成員19抗體
別    名 DNC; MCPHA; Mitochondrial thiamine pyrophosphate carrier; Mitochondrial uncoupling protein 1; MUP1; Solute carrier family 25 (mitochondrial deoxynucleotide carrier) member 19; Solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier) member 19; Solute carrier family 25 member 19; TPC.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 腫瘤  細胞生物  信號轉導  轉運蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, )
產品應用 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 36kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SLC25A19
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.
產品介紹 background:
This gene encodes a mitochondrial protein that is a member of the solute carrier family. Although this protein was initially thought to be the mitochondrial deoxynucleotide carrier involved in the uptake of deoxynucleotides into the matrix of the mitochondria, further studies have demonstrated that this protein instead functions as the mitochondrial thiamine pyrophosphate carrier, which transports thiamine pyrophosphates into mitochondria. Mutations in this gene cause microcephaly, Amish type, a metabolic disease that results in severe congenital microcephaly, severe 2-ketoglutaric aciduria, and death within the first year. Multiple alternatively spliced variants, encoding the same protein, have been identified for this gene. [provided by RefSeq, Jul 2008]

Function:
Mitochondrial transporter mediating uptake of thiamine pyrophosphate (ThPP) into mitochondria.

Tissue Specificity:
Expressed in all tissues examined except for placenta. Highest levels in colon, kidney, lung, testis, spleen, and brain.

DISEASE:
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A disorder characterized by severe congenital microcephaly and severe 2-ketoglutaric aciduria leading to death within the first year.
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A disease characterized by recurrent episodes of flaccid paralysis and encephalopathy associated with bilateral striatal necrosis and chronic progressive polyneuropathy.

Similarity:
Belongs to the mitochondrial carrier (TC 2.A.29) family.
Contains 3 Solcar repeats.

Database links:

Entrez Gene: 60386 Human

Omim: 606521 Human

SwissProt: Q9HC21 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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