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货号： bs-21224R-AF488 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-21224R-AF488
- 英文名称
- Anti-SLC25A22/AF488
- 中文名称
- AF488标记的溶质载体家族25成员22抗体
- 别 名
- GC 1; GC-1; GC1; GHC1_HUMAN; Glutamate/H(+) symporter 1; Mitochondrial glutamate carrier 1; SLC25A22; Solute carrier family 25 member 22.
- 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 信号转导 转运蛋白
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 4kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human SLC25A22
- 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:
This gene encodes a mitochondrial glutamate carrier. Mutations in this gene are associated with early infantile epileptic encephalopathy. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jul 2010]
Function:
nvolved in the transport of glutamate across the inner mitochondrial membrane. Glutamate is cotransported with H(+).
Subcellular Location:
Mitochondrion inner membrane.
Tissue Specificity:
Highly expressed in most tissues.
DISEASE:
Defects in SLC25A22 are the cause of epileptic encephalopathy early infantile type 3 (EIEE3) [MIM:609304]; also known as early myoclonic encephalopathy (EME) or neonatal epilepsy with suppression-burst pattern. Severe neonatal epilepsies with suppression-burst pattern are early-onset epileptic syndromes characterized by a typical EEG pattern. The suppression-burst pattern of the EEG is characterized by higher-voltage bursts of slow waves mixed with multifocal spikes alternating with isoelectric suppression phases. EME is characterized by a very early onset, erratic and fragmentary myoclonus, massive myoclonus, partial motor seizures and late tonic spasms. The prognosis of EME is poor, with no effective treatment, and children with the condition either die within 1 to 2 years after birth or survive in a persistent vegetative state. EME inheritance is autosomal recessive.
Similarity:
Belongs to the mitochondrial carrier family.
Contains 3 Solcar repeats.
Database links:Entrez Gene: 504371Cow
Entrez Gene: 79751Human
Entrez Gene: 68267Mouse
Entrez Gene: 309111Rat
Omim: 609302Human
SwissProt: Q08DK4Cow
SwissProt: Q9H936Human
SwissProt: Q9D6M3Mouse
Unigene: 16050Cow
Unigene: 99486Human
Unigene: 33729Mouse
Unigene: 98367Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.