FITC标记的线粒体二羧酸载体蛋白20抗体

¥2980
LMAI Bio
中国/美国/欧洲
2021-09-20 08:54

上海联迈生物工程有限公司

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产品属性
供应商上海联迈生物工程有限公司
数量大量
靶点详见说明书
级别1
目录编号LM-4192R-FITC
克隆性多克隆
抗原来源Rabbit
保质期1年
抗体英文名Anti-SLC25A20/FITC
抗体名Anti-SLC25A20/FITC
标记物FITC标记
宿主Human, Mouse, Rat, Cow, Horse, Rabbit,
适应物种Human, Mouse, Rat, Cow, Horse, Rabbit,
免疫原详见说明书
亚型IGg
形态粉末、液体、冻干粉
应用范围IF=1:50-200
浓度1mg/ml
保存条件-20 °C
规格100ul
产品说明
FITC标记的线粒体二羧酸载体蛋白20抗体
英文名称Anti-SLC25A20/FITC
中文名称FITC标记的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.
规格价格100ul/2980元 购买 大包装/询价
说 明 书100ul
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
交叉反应Human, Mouse, Rat, Cow, Horse, Rabbit,
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33kDa
性 状Lyophilized or Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human SLC25A20
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:
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. 

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.