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Mouse Anti-DNAJB6 Recombinant Antibody (1035) (V2LY-0425-LY639)


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Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
1035
Antibody Isotype
IgG2a, κ
Application
WB, IP, IF, ELISA

Basic Information

Immunogen
Amino acids 110-135 within an internal region of human DnaJB6.
Host Species
Mouse
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)1:50-1:500
ELISA1:100-1:1,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
Gelatin & PBS
Preservative
Sodium Azide
Concentration
0.2 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
DnaJ Heat Shock Protein Family (Hsp40) Member B6
Entrez Gene ID
Human10049
Mouse23950
Rat362293
UniProt ID
HumanO75190
MouseO54946
RatQ6AYU3
Function
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.
Biological Process
Actin cytoskeleton organization Source: Ensembl
Chorio-allantoic fusion Source: Ensembl
Chorion development Source: Ensembl
Extracellular matrix organization Source: Ensembl
Intermediate filament organization Source: UniProtKB
Negative regulation of cysteine-type endopeptidase activity involved in apoptotic process Source: UniProtKB
Negative regulation of inclusion body assembly Source: UniProtKB
Negative regulation of transcription, DNA-templated Source: Ensembl
Protein folding Source: UniProtKB
Protein localization to nucleus Source: Ensembl
Regulation of cellular response to heat Source: Reactome
Regulation of protein localization Source: ParkinsonsUK-UCL
Syncytiotrophoblast cell differentiation involved in labyrinthine layer development Source: Ensembl
Cellular Location
Nucleus; Perinuclear region; Z line
Involvement in disease
Muscular dystrophy, limb-girdle, autosomal dominant 1 (LGMDD1):
The disease is caused by variants affecting the gene represented in this entry. There is evidence that LGMDD1 is caused by dysfunction of isoform B (PubMed:22366786). 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.
More Infomation

Österlund, N., Lundqvist, M., Ilag, L. L., Gräslund, A., & Emanuelsson, C. (2020). Amyloid-β oligomers are captured by the DNAJB6 chaperone: Direct detection of interactions that can prevent primary nucleation. Journal of Biological Chemistry, 295(24), 8135-8144.

Palmio, J., Jonson, P. H., Inoue, M., Sarparanta, J., Bengoechea, R., Savarese, M., ... & Udd, B. (2020). Mutations in the J domain of DNAJB6 cause dominant distal myopathy. Neuromuscular Disorders, 30(1), 38-46.

Thiruvalluvan, A., de Mattos, E. P., Brunsting, J. F., Bakels, R., Serlidaki, D., Barazzuol, L., ... & Kampinga, H. H. (2020). DNAJB6, a key factor in neuronal sensitivity to amyloidogenesis. Molecular cell, 78(2), 346-358.

Rodríguez-González, C., Lin, S., Arkan, S., & Hansen, C. (2020). Co-chaperones DNAJA1 and DNAJB6 are critical for regulation of polyglutamine aggregation. Scientific reports, 10(1), 1-9.

Jiang, B., Zhao, Y., Shi, M. O., Song, L., Wang, Q., Qin, Q., ... & Liu, X. (2020). DNAJB6 promotes ferroptosis in esophageal squamous cell carcinoma. Digestive diseases and sciences, 65(7), 1999-2008.

Zhang, L., Zhou, Q., Qiu, Q., Hou, L., Wu, M., Li, J., ... & Lu, Y. (2019). CircPLEKHM3 acts as a tumor suppressor through regulation of the miR-9/BRCA1/DNAJB6/KLF4/AKT1 axis in ovarian cancer. Molecular cancer, 18(1), 1-19.

Månsson, C., Van Cruchten, R. T., Weininger, U., Yang, X., Cukalevski, R., Arosio, P., ... & Emanuelsson, C. (2018). Conserved S/T residues of the human chaperone DNAJB6 are required for effective inhibition of Aβ42 amyloid fibril formation. Biochemistry, 57(32), 4891-4902.

Jonson, P. H., Palmio, J., Johari, M., Penttilä, S., Evilä, A., Nelson, I., ... & Udd, B. (2018). Novel mutations in DNAJB6 cause LGMD 1D and distal myopathy in French families. European Journal of Neurology, 25(5), 790-794.

Aprile, F. A., Källstig, E., Limorenko, G., Vendruscolo, M., Ron, D., & Hansen, C. (2017). The molecular chaperones DNAJB6 and Hsp70 cooperate to suppress α-synuclein aggregation. Scientific reports, 7(1), 1-11.

Tsai, P. C., Tsai, Y. S., Soong, B. W., Huang, Y. H., Wu, H. T., Chen, Y. H., ... & Lee, Y. C. (2017). A novel DNAJB6 mutation causes dominantly inherited distal‐onset myopathy and compromises DNAJB6 function. Clinical Genetics, 92(2), 150-157.

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For research use only. Not intended for any clinical use.

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