Entry - *602634 - DNAJ/HSP40 HOMOLOG, SUBFAMILY B, MEMBER 9; DNAJB9 - OMIM
 
* 602634

DNAJ/HSP40 HOMOLOG, SUBFAMILY B, MEMBER 9; DNAJB9


Alternative titles; symbols

MICROVASCULAR ENDOTHELIAL DIFFERENTIATION GENE 1; MDG1
ERDJ4


HGNC Approved Gene Symbol: DNAJB9

Cytogenetic location: 7q31.1     Genomic coordinates (GRCh38): 7:108,569,874-108,574,850 (from NCBI)


TEXT

Gene Function

Prols et al. (1997) stated that expression of Mdg1 is increased during the tube-forming process of rat microvascular endothelial cells.

Van Galen et al. (2014) showed that human hematopoietic stem cells (HSCs) are predisposed to apoptosis through strong activation of the PERK (see EIF2AK3, 604032) branch of the unfolded protein response (UPR) after endoplasmic reticulum (ER) stress, whereas closely related progenitors exhibit an adaptive response leading to their survival. Enhanced ER protein folding by overexpression of the cochaperone ERDJ4 increased HSC repopulation capacity in xenograft assays, linking the UPR to HSC function. Because the UPR is a focal point where different sources of stress converge, van Galen et al. (2014) concluded that their study provided a framework for understanding how stress signaling is coordinated within tissue hierarchies and integrated with stemness.


Mapping

Prols et al. (1997) mapped the Mdg1 gene to rat chromosome 6q16-q23 by fluorescence in situ hybridization. They used the same method to map the human homolog, MDG1, to chromosome 14q24.2-q24.3. However, Gross (2011) mapped the DNAJB9 gene to chromosome 7q31.1 based on an alignment of the DNAJB9 sequence (GenBank AF083247) with the genomic sequence (GRCh37).


REFERENCES

  1. Gross, M. B. Personal Communication. Baltimore, Md. 2/17/2011.

  2. Prols, F., Liehr, T., Rinke, R., Rautenstrauss, B. Assignment of the microvascular endothelial differentiation gene 1 (MDG1) to human chromosome band 14q24.2-q24.3 by fluorescence in situ hybridization. Cytogenet. Cell Genet. 79: 149-150, 1997. [PubMed: 9533036, related citations] [Full Text]

  3. van Galen, P., Kreso, A., Mbong, N., Kent, D. G., Fitzmaurice, T., Chambers, J. E., Xie, S., Laurenti, E., Hermans, K., Eppert, K., Marciniak, S. J., Goodall, J. C., Green, A. R., Wouters, B. G., Wienholds, E., Dick, J. E. The unfolded protein response governs integrity of the haematopoietic stem-cell pool during stress. Nature 510: 268-272, 2014. [PubMed: 24776803, related citations] [Full Text]


Ada Hamosh - updated : 07/17/2014
Matthew B. Gross - updated : 2/17/2011
Creation Date:
Victor A. McKusick : 5/18/1998
alopez : 07/17/2014
mgross : 2/17/2011
carol : 8/17/2007
mgross : 7/31/2003
carol : 3/13/2001
carol : 5/18/1998

* 602634

DNAJ/HSP40 HOMOLOG, SUBFAMILY B, MEMBER 9; DNAJB9


Alternative titles; symbols

MICROVASCULAR ENDOTHELIAL DIFFERENTIATION GENE 1; MDG1
ERDJ4


HGNC Approved Gene Symbol: DNAJB9

Cytogenetic location: 7q31.1     Genomic coordinates (GRCh38): 7:108,569,874-108,574,850 (from NCBI)


TEXT

Gene Function

Prols et al. (1997) stated that expression of Mdg1 is increased during the tube-forming process of rat microvascular endothelial cells.

Van Galen et al. (2014) showed that human hematopoietic stem cells (HSCs) are predisposed to apoptosis through strong activation of the PERK (see EIF2AK3, 604032) branch of the unfolded protein response (UPR) after endoplasmic reticulum (ER) stress, whereas closely related progenitors exhibit an adaptive response leading to their survival. Enhanced ER protein folding by overexpression of the cochaperone ERDJ4 increased HSC repopulation capacity in xenograft assays, linking the UPR to HSC function. Because the UPR is a focal point where different sources of stress converge, van Galen et al. (2014) concluded that their study provided a framework for understanding how stress signaling is coordinated within tissue hierarchies and integrated with stemness.


Mapping

Prols et al. (1997) mapped the Mdg1 gene to rat chromosome 6q16-q23 by fluorescence in situ hybridization. They used the same method to map the human homolog, MDG1, to chromosome 14q24.2-q24.3. However, Gross (2011) mapped the DNAJB9 gene to chromosome 7q31.1 based on an alignment of the DNAJB9 sequence (GenBank AF083247) with the genomic sequence (GRCh37).


REFERENCES

  1. Gross, M. B. Personal Communication. Baltimore, Md. 2/17/2011.

  2. Prols, F., Liehr, T., Rinke, R., Rautenstrauss, B. Assignment of the microvascular endothelial differentiation gene 1 (MDG1) to human chromosome band 14q24.2-q24.3 by fluorescence in situ hybridization. Cytogenet. Cell Genet. 79: 149-150, 1997. [PubMed: 9533036] [Full Text: https://doi.org/10.1159/000134706]

  3. van Galen, P., Kreso, A., Mbong, N., Kent, D. G., Fitzmaurice, T., Chambers, J. E., Xie, S., Laurenti, E., Hermans, K., Eppert, K., Marciniak, S. J., Goodall, J. C., Green, A. R., Wouters, B. G., Wienholds, E., Dick, J. E. The unfolded protein response governs integrity of the haematopoietic stem-cell pool during stress. Nature 510: 268-272, 2014. [PubMed: 24776803] [Full Text: https://doi.org/10.1038/nature13228]


Contributors:
Ada Hamosh - updated : 07/17/2014
Matthew B. Gross - updated : 2/17/2011

Creation Date:
Victor A. McKusick : 5/18/1998

Edit History:
alopez : 07/17/2014
mgross : 2/17/2011
carol : 8/17/2007
mgross : 7/31/2003
carol : 3/13/2001
carol : 5/18/1998