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产品信息
H00009444-B01P
产品规格
种属反应
Human
宿主/亚型
Mouse
/ IgG
分类
Polyclonal
类型
Antibody
抗原
QKI (NP_996735, 1 a.a. approximately 319 a.a) full-length human protein.
偶联物
形式
Liquid
浓度
0.5-1 mg/mL
纯化类型
Protein A
保存液
PBS, pH 7.4
内含物
no preservative
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Ambient (domestic); Wet ice (international)
产品详细信息
Product may be used with Western Blot (Transfected lysate).
The Isotype of this product is composed of an IgG Mixture.
Immunogen sequence: MVGEMETKEK PKPTPDYLMQ LMNDKKLMSS LPNFCGIFNH LERLLDEEIS RVRKDMYNDT LNGSTEKRSA ELPDAVGPIV QLQEKLYVPV KEYPDFNFVG RILGPRGLTA KQLEAETGCK IMVRGKGSMR DKKKEEQNRG KPNWEHLNED LHVLITVEDA QNRAEIKLKR AVEEVKKLLV PAAEGEDSLK KMQLMELAIL NGTYRDANIK SPALAFSLAA TAQAAPRIIT GPAPVLPPAA LRTPTPAGPT IMPLIRQIQT AVMPNGTPHP TAAIVPPGPE AGLIYTPYEY PYTLAPATSI LEYPIEPSGV LGMAFPTKG
靶标信息
This gene encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. This gene includes a very large exon spanning more than 12.8 kb. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. Sacsin has been found to protect against mutant ataxin-1. A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants.
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: DKFZp586I0923; homolog of mouse quaking QKI (KH domain RNA binding protein); Hqk; KH domain-containing RNA-binding protein QKI; Protein quaking; QKI, KH domain containing, RNA binding; quaking homolog, KH domain RNA binding; RNA Binding (QKI); RNA binding protein HQK
基因别名: HKQ; Hqk; hqkI; QK; QK1; QK3; QKI
UniProt ID: (Human) Q96PU8
Entrez Gene ID: (Human) 9444
