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AFG3樣蛋白2/脊髓小腦共濟(jì)失調(diào)蛋白28抗體
  • 產(chǎn)品貨號(hào)：
    BN40325R
  • 中文名稱：
    AFG3樣蛋白2/脊髓小腦共濟(jì)失調(diào)蛋白28抗體
  • 英文名稱：
    Rabbit anti-AFG3L2 Polyclonal antibody
  • 品牌：
    Biorigin

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  • BN40325R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Mouse,Rat(predicted:Human,Chicken,Dog,Pig,Cow,Horse,Sheep) 推薦應(yīng)用：IHC-P,IHC-F,ICC,IF,ELISA

  • BN40325R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Mouse,Rat(predicted:Human,Chicken,Dog,Pig,Cow,Horse,Sheep) 推薦應(yīng)用：IHC-P,IHC-F,ICC,IF,ELISA

產(chǎn)品描述

英文名稱AFG3L2
中文名稱AFG3樣蛋白2/脊髓小腦共濟(jì)失調(diào)蛋白28抗體
別    名SCA28; AFG3 (ATPase family gene 3, yeast) like 2; AFG3 ATPase family gene 3 like 2 (yeast); AFG3 ATPase family gene 3 like 2; AFG3 like protein 2; ATPase family gene 3 like 2; ATPase family gene 3 yeast; FLJ25993; Paraplegin like protein; SCA28; Spinocerebellar ataxia 28; AFG32_HUMAN .  
研究領(lǐng)域腫瘤  細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  泛素  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量89kDa
細(xì)胞定位細(xì)胞膜 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human AFG3L2:531-600/797 
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Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹AFG3L2 is a multi-pass membrane metalloprotease that contains one AAA (ATPase associated with diverse cellular activities) domain, a zinc-dependent binding motif, an RNA-binding region and an ATP/GTP binding site. Localizing to the mitochondrial membrane, AFG3L2 is ubiquitously expressed with highest expression levels in skeletal muscle and heart. AFG3L2 shares 69% similarity with the yeast Afg3 protein and 49% similarity with Paraplegin, a protein of mitochondria that is thought to be involved in signal transduction and chaperone-like activities. In mitochondria, AFG3L2 forms a complex with Paraplegin that is believed to regulate essential protein quality control. Mutations in the gene encoding either one of these proteins can result in hereditary spastic paraplegia, a degenerative spinal cord disorder that is characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence.

Function:
AFG3L2 is a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. AFG3L2 is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders and is a putative ATP dependent protease

Subunit:
Homooligomer. Interacts with SPG7; the interaction is required for the efficient assembly of mitochondrial complex I.

Subcellular Location:
Mitochondrial membrane; multipass membrane protein

Tissue Specificity:
Ubiquitous. Highly expressed in the cerebellar Purkinje cells.

DISEASE:
Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) [MIM:610246]. It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment.
Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5) [MIM:614487]. A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.

Similarity:
In the N-terminal section; belongs to the AAA ATPase family.
In the C-terminal section; belongs to the peptidase M41 family.

SWISS:
Q9Y4W6

Gene ID:
10939

Database links:

Entrez Gene: 10939 Human

Omim: 604581 Human

SwissProt: Q9Y4W6 Human

Unigene: 726355 Human



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