Human DnaJ (Hsp40) homolog, subfamily B, member 6 ELISA Kit | DNAJB6 elisa kit
Human DnaJ homolog subfamily B member 6, DNAJB6 ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the DNAJ protein family. DNAJ family members are characterized by a highly conserved amino acid stretch called the 'J-domain' and function as one of the two major classes of molecular chaperones involved in a wide range of cellular events, such as protein folding and oligomeric protein complex assembly. This family member may also play a role in polyglutamine aggregation in specific neurons. Alternative splicing of this gene results in multiple transcript variants; however, not all variants have been fully described. [provided by RefSeq, Jul 2008]
Uniprot Description
DNAJB6: Plays an indispensable role in the organization of KRT8/KRT18 filaments. Acts as an endogenous molecular chaperone for neuronal proteins including huntingtin. Suppresses aggregation and toxicity of polyglutamine-containing, aggregation-prone proteins. Isoform B but not isoform A inhibits huntingtin aggregation. Has a stimulatory effect on the ATPase activity of HSP70 in a dose-dependent and time-dependent manner and hence acts as a co-chaperone of HSP70. Also reduces cellular toxicity and caspase-3 activity. Defects in DNAJB6 are the cause of limb-girdle muscular dystrophy type 1E (LGMD1E). An autosomal dominant myopathy characterized by adult onset of proximal muscle weakness, beginning in the hip girdle region and later progressing to the shoulder girdle region. There is evidence that LGMD1E is caused by dysfunction of isoform B (PubMed:22366786). 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Chaperone
Chromosomal Location of Human Ortholog: 7q36.3
Cellular Component: nucleoplasm; membrane; perinuclear region of cytoplasm; cytoplasm; Z disc; cytosol; nucleus
Molecular Function: protein binding; ATPase activator activity; heat shock protein binding; chaperone binding; unfolded protein binding
Biological Process: protein folding; positive regulation of ATPase activity; intermediate filament organization; negative regulation of caspase activity
Disease: Muscular Dystrophy, Limb-girdle, Type 1e
Research Articles on DNAJB6
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Product Notes
The Human DNAJB6 dnajb6 (Catalog #AAA9340752) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9340752 ELISA Kit recognizes Human DNAJB6. It is sometimes possible for the material contained within the vial of "DnaJ (Hsp40) homolog, subfamily B, member 6, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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