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货号： bs-8428R-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8428R-Cy3
- 英文名称
- Anti-BRP44L/Cy3
- 中文名称
- Cy3标记的脑蛋白44样蛋白抗体
- 别 名
- Apoptosis regulating basic protein; Brain protein 44 like protein; CGI129; HSPC040; PNAS115; MPC1_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 神经生物学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep,
- 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
- 12kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human BRP44L
- 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:
BRP44L, also known as HSPC040 or CGI-129, is a 109 amino acid mitochondrial protein belonging to the UPF0041 family. The gene that encodes BRP44L maps to human chromosome 6. Making up nearly 6% of the human genome, chromosome 6 contains around 1,200 genes within 170 million base pairs of sequence. Deletion of a portion of the q arm of chromosome 6 is associated with early onset intestinal cancer suggesting the presence of a cancer susceptibility locus. Porphyria cutanea tarda is associated with chromosome 6 through the HFE gene which, when mutated, predisposes an individual to developing this porphyria. Notably, the PARK2 gene, which is associated with Parkinsons disease, and the genes encoding the major histocompatiblity complex proteins, which are key molecular components of the immune system and determine predisposition to rheumatic diseases, are also located on chromosome 6. Stickler syndrome, 21-hydroxylase deficiency and maple syrup urine disease are also associated with genes on chromosome 6. A bipolar disorder susceptibility locus has been identified on the q arm of chromosome 6.
Function:
Mediates the uptake of pyruvate into mitochondria.
Subunit:
The functional 150 kDa pyruvate import complex is a heteromer of MPC1 and MPC2 (By similarity).
Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.
DISEASE:
Defects in MPC1 are the cause of mitochondrial pyruvate carrier deficiency (MPYCD) [MIM:614741]. An autosomal recessive metabolic disorder characterized by severely delayed psychomotor development, mild dysmorphic features, hepatomegaly, marked metabolic acidosis, hyperlactacidemia with normal lactate/pyruvate, and encephalopathy. Some patients have epilepsy and peripheral neuropathy.
Similarity:
Belongs to the mitochondrial pyruvate carrier (MPC) family.
Database links:
UniProtKB/Swiss-Prot: Q9Y5U8.1
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.