DNAJB6 Antibody (PACO48502)
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주문- SKU:
- PACO48502
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | DNAJB6 Antibody (PACO48502) |
Antibody SKU: | PACO48502 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:2000-1:10000, IF:1:50-1:200 |
Species Reactivity: | Human, Mouse |
Immunogen: | Recombinant Human DnaJ homolog subfamily B member 6 protein (208-326AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western Blot. Positive WB detected in: Mouse skeletal muscle tissue. All lanes: DNAJB6 antibody at 2.8µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 37, 27, 36, 31 kDa. Observed band size: 37 kDa.. | |
Immunofluorescent analysis of HepG2 cells using PACO48502 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
Background: | 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. |
Synonyms: | DnaJ homolog subfamily B member 6 (HHDJ1) (Heat shock protein J2) (HSJ-2) (MRJ) (MSJ-1), DNAJB6, HSJ2 MRJ MSJ1 |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Chaperone Chromosomal Location of Human Ortholog: 7q36.3 Cellular Component: cytosol; membrane; nucleoplasm; nucleus; perinuclear region of cytoplasm; Z disc Molecular Function:ATPase activator activity; chaperone binding; heat shock protein binding; protein binding; unfolded protein binding Biological Process: intermediate filament organization; negative regulation of caspase activity; protein folding; regulation of protein localization Disease: Muscular Dystrophy, Limb-girdle, Type 1e |
NCBI Summary: | 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 Code: | O75190 |
NCBI GenInfo Identifier: | 19855067 |
NCBI Gene ID: | 10049 |
NCBI Accession: | O75190.2 |
UniProt Secondary Accession: | O75190,O95806, Q53EN8, Q59EF2, Q6FIC8, Q75MA2, Q9UIK6 A4D232, A8K7D8, A8KAG0, B4DN73, E9PCZ2, |
UniProt Related Accession: | O75190 |
Molecular Weight: | 30,369 Da |
NCBI Full Name: | DnaJ homolog subfamily B member 6 |
NCBI Synonym Full Names: | DnaJ heat shock protein family (Hsp40) member B6 |
NCBI Official Symbol: | DNAJB6 |
NCBI Official Synonym Symbols: | DJ4; MRJ; DnaJ; HSJ2; HHDJ1; HSJ-2; MSJ-1; LGMD1D; LGMD1E |
NCBI Protein Information: | dnaJ homolog subfamily B member 6 |
UniProt Protein Name: | DnaJ homolog subfamily B member 6 |
UniProt Synonym Protein Names: | HHDJ1; Heat shock protein J2; HSJ-2; MRJ; MSJ-1 |
Protein Family: | DnaJ homolog subfamily |
UniProt Gene Name: | DNAJB6 |
UniProt Entry Name: | DNJB6_HUMAN |