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产品信息
PA5-88901
产品规格
种属反应
Human,
Mouse
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant fusion protein containing a sequence corresponding to amino acids 1-361 of human Ataxin-3 (ATXN3) (NP_004984.2).
偶联物
形式
Liquid
浓度
0.15 mg/mL
纯化类型
Affinity Chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
AB_2805206
产品详细信息
Immunogen sequence: MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQMHRPK LIGEELAQLK EQRVHKTDLE RVLEANDGSG MLDEDEEDLQ RALALSRQEI DMEDEEADLR RAIQLSMQGS SRNISQDMTQ TSGTNLTSEE LRKRREAYFE KQQQKQQQQQ QQQQQGDLSG QSSHPCERPA TSSGALGSDL GDAMSEEDML QAAVTMSLET VRNDLKTEGK K; Positive Samples: MCF7, SW480, Jurkat, A-549, DU145, Mouse brain, Mouse liver; Cellular Location: Nucleus matrix
靶标信息
Machado-Joseph disease is an autosomal dominant neurologic disorder, and is now known to be the same as previously described spinocerebellar ataxia-3. MJD protein (Ataxin-3) contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. This protein interacts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription, and also acts as a histone-binding protein that regulates transcription. MJD is a deubiquitinating enzyme.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: ataxin 3 variant an; ataxin 3 variant ao; ataxin 3 variant at; ataxin 3 variant e; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant r; ataxin 3 variant ref; ataxin 3 variant y; Ataxin-3; ataxin3; josephin; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) homolog; Machado-Joseph disease protein 1; Machado-Joseph disease protein 1 homolog; olivopontocerebellar ataxia 3; spin; Spinocerebellar ataxia type 3 protein
基因别名: 2210008M02Rik; AI463012; AI647473; AT3; ataxin-3; ATX3; ATXN3; JOS; MJD; MJD1; SCA3
UniProt ID: (Human) P54252, (Mouse) Q9CVD2
Entrez Gene ID: (Human) 4287, (Mouse) 110616
RNA polymerase II regulatory region DNA binding
histone deacetylase activity
thiol-dependent ubiquitin-specific protease activity
protein binding
omega peptidase activity
ubiquitin protein ligase binding
identical protein binding
ATPase binding
Lys63-specific deubiquitinase activity
Lys48-specific deubiquitinase activity
peptidase activity
cysteine-type peptidase activity
hydrolase activity
chromatin/chromatin-binding, or -regulatory protein
DNA metabolism protein
microtubule cytoskeleton organization
nucleotide-excision repair
transcription, DNA-templated
regulation of transcription, DNA-templated
ubiquitin-dependent protein catabolic process
misfolded or incompletely synthesized protein catabolic process
chemical synaptic transmission
nervous system development
regulation of cell-substrate adhesion
protein deubiquitination
actin cytoskeleton organization
cellular response to heat
monoubiquitinated protein deubiquitination
exploration behavior
proteasome-mediated ubiquitin-dependent protein catabolic process
intermediate filament cytoskeleton organization
protein K63-linked deubiquitination
histone H3 deacetylation
protein K48-linked deubiquitination
cellular response to misfolded protein
positive regulation of ERAD pathway
protein localization to cytosolic proteasome complex involved in ERAD pathway
proteolysis
