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RabbitAnti-BRP44L Rabbit pAb  antibody (bs-8428R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-8428R
英文名称 BRP44L Rabbit pAb
中文名称 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.  
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
克 隆 号
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep, )
产品应用 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 12 kDa
检测分子量
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BRP44L: 1-80/109 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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 Parkinson's 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.

SWISS:
Q9Y5U8

Gene ID:
51660

Database links:

Entrez Gene: 51660 Human

Entrez Gene: 55951 Mouse

Entrez Gene: 171087 Rat

Omim: 614738 Human

SwissProt: Q9Y5U8 Human

SwissProt: P63030 Mouse

SwissProt: P63031 Rat

Unigene: 172755 Human

Unigene: 288510 Mouse

Unigene: 3718 Rat



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