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Anti-AFG3L2/FITC抗体
产品编号YB-11704R-FITC
英文名称Anti-AFG3L2/FITC
中文名称FITC标记的AFG3样蛋白2/脊髓小脑共济失调蛋白28抗体
别 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 .
Anti-AFG3L2/FITC抗体
说 明 书100ul
研究领域肿瘤 细胞生物 神经生物学 信号转导 泛素
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
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89kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human AFG3L2 (531-600aa)
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
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.
Anti-AFG3L2/FITC抗体
产品介绍background:
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.Anti-AFG3L2/FITC抗体 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.
Anti-AFG3L2/FITC抗体Similarity:
In the N-terminal section; belongs to the AAA ATPase family.
In the C-terminal section; belongs to the peptidase M41 family.
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.