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Rabbit Anti-SLC25A22/BF488 Conjugated antibody (bs-21224R-BF488)
訂購(gòu)熱線:400-901-9800
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-21224R-BF488
英文名稱1 Rabbit Anti-SLC25A22/BF488 Conjugated antibody
中文名稱 BF488標(biāo)記的溶質(zhì)載體家族25成員22抗體
別    名 GC 1; GC-1; GC1; GHC1_HUMAN; Glutamate/H(+) symporter 1; Mitochondrial glutamate carrier 1; SLC25A22; Solute carrier family 25 member 22.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  轉(zhuǎn)運(yùn)蛋白  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, )
產(chǎn)品應(yīng)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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SLC25A22
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 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: 504371 Cow

Entrez Gene: 79751 Human

Entrez Gene: 68267 Mouse

Entrez Gene: 309111 Rat

Omim: 609302 Human

SwissProt: Q08DK4 Cow

SwissProt: Q9H936 Human

SwissProt: Q9D6M3 Mouse

Unigene: 16050 Cow

Unigene: 99486 Human

Unigene: 33729 Mouse

Unigene: 98367 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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