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DNAJC21

Function

May act as a co-chaperone for HSP70. May play a role in ribosomal RNA (rRNA) biogenesis, possibly in the maturation of the 60S subunit. Binds the precursor 45S rRNA.

Involvement in disease

Bone marrow failure syndrome 3

BMFS3

A form of bone marrow failure syndrome, a heterogeneous group of life-threatening disorders characterized by hematopoietic defects in association with a range of variable extra-hematopoietic manifestations. BMFS3 is characterized by pancytopenia with onset in early childhood. Some patients have additional variable non-specific features, including poor growth, microcephaly, and skin anomalies. BMFS3 inheritance is autosomal recessive.

None

The disease is caused by variants affecting the gene represented in this entry.

Tissue Specificity

Expressed in brain, placenta, kidney and pancreas.

Cellular localization

Alternative names

DNAJA5, DNAJC21, DnaJ homolog subfamily C member 21, DnaJ homolog subfamily A member 5, Protein GS3

swissprot:Q5F1R6 entrezGene:134218