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Rabbit Anti-DNAJB11 Recombinant Antibody (EG976) (CBMAB-EN1158-LY)

The product is antibody recognizes DNAJB11. The antibody EG976 immunoassay techniques such as: WB: 1:500~1:1000 IHC: 1:50~1:100 ELISA: 1:40000.
See all DNAJB11 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
EG976
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 IHC: 1:50~1:100 ELISA: 1:40000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human DNAJB11.
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format
Liquid
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 B11
Introduction
This gene encodes a soluble glycoprotein of the endoplasmic reticulum (ER) lumen that functions as a co-chaperone of binding immunoglobulin protein, a 70 kilodalton heat shock protein chaperone required for the proper folding and assembly of proteins in the ER. The encoded protein contains a highly conserved J domain of about 70 amino acids with a characteristic His-Pro-Asp (HPD) motif and may regulate the activity of binding immunoglobulin protein by stimulating ATPase activity. [provided by RefSeq, Mar 2014]
Entrez Gene ID
Human51726
Mouse67838
Rat360734
UniProt ID
HumanQ9UBS4
MouseQ99KV1
RatQ6TUG0
Alternative Names
DnaJ Heat Shock Protein Family (Hsp40) Member B11; Endoplasmic Reticulum DNA J Domain-Containing Protein 3; DnaJ (Hsp40) Homolog, Subfamily B, Member 11; ER-Associated Hsp40 Co-Chaperone; PWP1-Interacting Protein 4; APOBEC1-Binding Protein 2; ER-Resident Protein ERdj3; ABBP-2; ERdj3; ERj3p; ERj3; EDJ; DnaJ Homolog Subfamily B Member 11; ER-Associated DNAJ Protein 3;
Function
As a co-chaperone for HSPA5 it is required for proper folding, trafficking or degradation of proteins (PubMed:10827079, PubMed:15525676, PubMed:29706351).

Binds directly to both unfolded proteins that are substrates for ERAD and nascent unfolded peptide chains, but dissociates from the HSPA5-unfolded protein complex before folding is completed (PubMed:15525676).

May help recruiting HSPA5 and other chaperones to the substrate. Stimulates HSPA5 ATPase activity (PubMed:10827079).

It is necessary for maturation and correct trafficking of PKD1 (PubMed:29706351).
Biological Process
Positive regulation of ATPase activity Source: AgBase
Protein folding Source: InterPro
Protein maturation Source: UniProtKB
Cellular Location
Endoplasmic reticulum lumen. Associated with the ER membrane in a C-terminally epitope-tagged construct.
Involvement in disease
Polycystic kidney disease 6 with or without polycystic liver disease (PKD6):
A form of polycystic kidney disease, a disorder characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occur in other organs, particularly the liver. PKD6 inheritance is autosomal dominant.
PTM
Contains high-mannose Endo H-sensitive carbohydrates.
Cys-169, Cys-171, Cys-193 and Cys-196 form intramolecular disulfide bonds. The preferential partner for each Cys is not known.
Thr-188 was reported to be phosphorylated upon DNA damage by ATM or ATR; however as this position has been shown to be in the ER lumen, the in vivo relevance is not proven.

Pisani, I., Allinovi, M., Palazzo, V., Zanelli, P., Gentile, M., Farina, M. T., ... & Manenti, L. (2022). More dissimilarities than affinities between DNAJB11-PKD and ADPKD. Clinical Kidney Journal.

Jordan, P., Arrondel, C., Bessières, B., Tessier, A., Attié-Bitach, T., Guterman, S., ... & Heidet, L. (2021). Bi-allelic pathogenic variations in DNAJB11 cause Ivemark II syndrome, a renal-hepatic-pancreatic dysplasia. Kidney International, 99(2), 405-409.

Sun, R., Yang, L., Wang, Y., Zhang, Y., Ke, J., & Zhao, D. (2021). DNAJB11 predicts a poor prognosis and is associated with immune infiltration in thyroid carcinoma: a bioinformatics analysis. Journal of International Medical Research, 49(11), 03000605211053722.

Huynh, V. T., Audrézet, M. P., Sayer, J. A., Ong, A. C., Lefevre, S., Le Brun, V., ... & Genomics England Research Consortium. (2020). Clinical spectrum, prognosis and estimated prevalence of DNAJB11-kidney disease. Kidney international, 98(2), 476-487.

Wilson, G. J., Wood, S., Patel, C., Oliver, K., John, G., Ranganathan, D., ... & Isbel, N. (2020). DNAJB11-related atypical ADPKD in a kidney transplant donor. Kidney international reports, 5(8), 1363-1366.

Pan, J., Cao, D., & Gong, J. (2018). The endoplasmic reticulum co-chaperone ERdj3/DNAJB11 promotes hepatocellular carcinoma progression through suppressing AATZ degradation. Future Oncology, 14(29), 3001-3013.

Cornec-Le Gall, E., Olson, R. J., Besse, W., Heyer, C. M., Gainullin, V. G., Smith, J. M., ... & Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease. (2018). Monoallelic mutations to DNAJB11 cause atypical autosomal-dominant polycystic kidney disease. The American Journal of Human Genetics, 102(5), 832-844.

Allison, S. J. (2018). DNAJB11: another player in ADPKD. Nature Reviews Nephrology, 14(8), 476-476.

Chen, K. C., Qu, S., Chowdhury, S., Noxon, I. C., Schonhoft, J. D., Plate, L., ... & Wiseman, R. L. (2017). The endoplasmic reticulum HSP 40 co‐chaperone ER dj3/DNAJB 11 assembles and functions as a tetramer. The EMBO journal, 36(15), 2296-2309.

Guorong, Y., Yunfeng, F. U., Yanli, L. I., Caiyun, Z., & Weiguo, L. V. (2017). Expression and clinical significance of DNAJB11 in epithelial ovarian cancer. Zhejiang da xue xue bao. Yi xue ban= Journal of Zhejiang University. Medical Sciences, 46(2), 173-178.

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

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