小脑脊髓共济失调蛋白3抗体

产品货号：

BN41018R
中文名称：

小脑脊髓共济失调蛋白3抗体
英文名称：

Rabbit anti-ATXN3L Polyclonal antibody
品牌：

Biorigin

货号

产品规格

售价

备注
BN41018R-100ul

100ul

¥2360.00

交叉反应：Human 推荐应用：WB,IHC-P,IHC-F,IF
BN41018R-200ul

200ul

¥3490.00

交叉反应：Human 推荐应用：WB,IHC-P,IHC-F,IF

产品描述

英文名称	ATXN3L
中文名称	小脑脊髓共济失调蛋白3抗体
别名	ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.
研究领域	细胞生物免疫学染色质和核信号表观遗传学泛素
抗体来源	Rabbit
克隆类型	Polyclonal
交叉反应	Human,
产品应用	WB=1:500-2000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
分子量	41kDa
细胞定位	细胞膜
性状	Liquid
浓度	1mg/ml
免疫原	KLH conjugated synthetic peptide derived from human ATXN3L:251-355/355
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	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed	PubMed
产品介绍	Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) ; also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia 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. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease. Function: Deubiquitinating enzyme that cleaves both 'Lys-48'-linked and 'Lys-63'-linked poly-ubiquitin chains (in vitro). Subcellular Location: Nucleus (By similarity). Similarity: Contains 1 Josephin domain. Contains 2 UIM (ubiquitin-interacting motif) repeats. SWISS: Q9H3M9 Gene ID: 25814 Database links: Entrez Gene: 25814 Human Entrez Gene: 170821 Rat Omim: 611150 Human SwissProt: Q9UBB4 Human SwissProt: Q9ER24 Rat Unigene: 475125 Human Unigene: 6524 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.