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SLC25A20 Rabbit pAb (bs-4192R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-4192R
英文名称 SLC25A20 Rabbit pAb
中文名称 线粒体二羧酸载体蛋白20抗体
别    名 CAC; CACT; Carnitine/acylcarnitine translocase; Solute carrier family 25 member 20; MCAT_HUMAN.  
研究领域 肿瘤  细胞生物  免疫学  染色质和核信号  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Mouse (predicted: Human,Rat,Rabbit,Cow,Horse)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 33 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SLC25A20: 101-200/301 
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 SLC25A20 is one of several closely related mitochondrial membrane carrier proteins that shuttle substrates between cytosol and the intramitochondrial matrix space. It mediates the transport of acylcarnitines into the mitochondrial matrix for their oxidation by the mitochondrial fatty acid oxidation pathway. Mutations in this gene are associated with carnitine acylcarnitine translocase deficiency, which can cause a variety of pathological conditions such as hypoglycemia, cardiac arrest, hepatomegaly, hepatic dysfunction and muscle weakness, and is usually lethal in new born and infants.

Function:
Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.

Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.

DISEASE:
Carnitine-acylcarnitine translocase deficiency (CACT deficiency) [MIM:212138]: A rare long-chain fatty acid oxidation disorder. Metabolic consequences include hypoketotic hypoglycemia under fasting conditions, hyperammonemia, elevated creatine kinase and transaminases, dicarboxylic aciduria, very low free carnitine and abnormal acylcarnitine profile with marked elevation of the long-chain acylcarnitines. Clinical features include neurologic abnormalities, cardiomyopathy, arrhythmias, skeletal muscle damage, liver dysfunction and episodes of life-threatening coma, which eventually lead to death. Most patients become symptomatic in the neonatal period with a rapidly progressive deterioration and a high mortality rate. Note=The disease is caused by mutations affecting the gene represented in this entry.

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

SWISS:
O43772

Gene ID:
788

Database links:

Entrez Gene: 788 Human

Entrez Gene: 57279 Mouse

Entrez Gene: 117035 Rat

Omim: 212138 Human

SwissProt: O43772 Human

SwissProt: Q9Z2Z6 Mouse

SwissProt: P97521 Rat

Unigene: 13845 Human

Unigene: 29666 Mouse

Unigene: 3289 Rat



产品图片
Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti-SLC25A20 (bs-4192R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 33 kD Observed band size: 33 kD
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