线粒体二羧酸载体蛋白20抗体
规格：1mg/1ml
英文名： SLC25A20
别名： CAC; CACT; Carnitine/acylcarnitine translocase; Solute carrier family 25 member 20; MCAT_HUMAN.
分子量： 33kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SLC25A20
交叉反应：Human, Mouse, Rat, Cow, Horse, Rabbit,
细胞定位：
线粒体二羧酸载体蛋白20抗体产品介绍：background: 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线粒体二羧酸载体蛋白20抗体 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线粒体二羧酸载体蛋白20抗体 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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
线粒体二羧酸载体蛋白20抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （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.
研究领域：肿瘤 细胞生物 **学 染色质和核信号 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid