Invitrogen™ QKI Monoclonal Antibody (N147/6)

Catalog No.	Quantity
PIMA527651	100 μg

Catalog No. PIMA527651 Supplier Invitrogen™ Supplier No. MA527651

Description

Mouse Monoclonal Antibody

1 μg/mL of MA5-27651 was sufficient for detection of Pan-QKI in 20 μg of rat brain lysate by colorimetric immunoblot analysis using Goat anti-mouse IgG:HRP as the secondary antibody. Detects approximately 36-38kDa. This antibody was formerly sold as clone S147-6.

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.

Specifications

• Antigen: QKI
• Applications: Immunohistochemistry (PFA fixed), Western Blot, Immunocytochemistry
• Classification: Monoclonal
• Clone: N147/6
• Concentration: 1 mg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 50% glycerol and 0.1% sodium azide; pH 7.4
• Gene: QKI
• Gene Accession No.: Q91XU1, Q96PU8, Q9QYS9
• Gene Alias: 1110003F05Rik; 1500005P18; DKFZp586I0923; HKQ; homolog of mouse quaking QKI (KH domain RNA binding protein); Hqk; hqkI; l(17)-1Wis; l17Wis1; lethal, Chr 17, U Wisconsin 1; MqkI; Protein quaking; protein quaking-like; Qk; QK1; QK3; Qka1; Qki; QKI, KH domain containing RNA binding; QKI, KH domain containing, RNA binding; QKI-5 protein; quaking; quaking homolog, KH domain RNA binding; RNA Binding (QKI); RNA binding protein HQK; rqkI
• Gene Symbols: Qk, QKI
• Host Species: Mouse
• Immunogen: Fusion protein amino acids 1-341 (full-length) of human QKI-5. Mouse: 100% identity (341/341 amino acids identical). Rat: 99% identity (339/341 amino acids identical) >90% identity with QKI-6, QKI-7 and QKI-7b.
• Purification Method: Protein G
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 100910212, 19317, 499022, 9444
• Target Species: Human, Mouse, Rat
• Content And Storage: -20°C
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG2b