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DNA repair protein RAD50; hRAD50 (RAD50)

Function: - component of the MRN complex, which plays a central role in double-strand break (DSB) repair, DNA recombination, maintenance of telomere integrity & meiosis - within the MRN complex, RAD50 may be required to bind DNA ends & hold them in close proximity; this could facilitate searches for short or long regions of sequence homology in the recombining DNA templates, & may also stimulate activity of DNA ligases &/or restrict nuclease activity of MRE11A to prevent nucleolytic degradation past a given point - phosphorylated upon DNA damage, probably by ATM or ATR - component of the BASC complex - found in a complex with TERF2 - interacts with RINT1 - interacts with BRCA1 via its N-terminal domain - interacts with DCLRE1C/Artemis Cofactor: binds 1 Zn+2 per homodimer (putative) Structure: - the Zn+2-hook, which separates the large intramolecular coiled coil regions, contains 2 Cys that coordinate one molecule of Zn+2 with the help of the 2 Cys of the Zn+2-hook of another RAD50 molecule, thereby forming a V-shaped homodimer - the two heads of the homodimer, which constitute the ATP-binding domain, interact with the MRE11A homodimer - belongs to the SMC family, RAD50 subfamily - contains 1 Zn+2-hook domain Compartment: - nucleus, telomere - localizes to discrete nuclear foci after treatment with genotoxic agents Alternative splicing: named isoforms=3 Expression: - expressed at very low level in most tissues, except in testis where it is expressed at higher level - expressed in fibroblasts

General

DNA-binding protein phosphoprotein zinc finger protein

Properties

SIZE: entity length = 1312 aa MW = 154 kD COMPARTMENT: cell nucleus MOTIF: ATP-binding site NAME: ATP-binding site SITE: 36-43 coiled coil {228-359} MOTIF: Ser phosphorylation site {S237} coiled coil {401-598} Zinc-hook {635-734} MOTIF: coiled coil {635-673} Ser phosphorylation site {S635} Ser phosphorylation site {S640} Zn+2-binding site SITE: 681-681 Zn+2-binding site SITE: 684-684 Thr phosphorylation site {T690} coiled coil {706-734} coiled coil {789-1079}

References

  1. Khanna KK & Jackson SP DNA double-strand breaks: signaling, repair and the cancer connection. Nature Genetics 27:247-54, 2001 PMID: 11242102
  2. UniProt :accession Q92878

Component-of

MRN complex

Databases & Figures

OMIM 604040 UniProt Q92878 PFAM correlations Entrez Gene 10111 Kegg hsa:10111 Figures/diagrams/slides/tables related to DNA repair protein RAD50