Ku86-deficient mice exhibit severe combined immunodeficiency and defective processing of V(D)J recombination intermediates

Ku is a heterodimeric DNA end binding complex composed of 70 and 86 kDa subunits. Here, we show that Ku86 is essential for normal V(D)J recombination in vivo, as Ku86-deficient mice are severely defective for formation of coding joints. Unlike severe combined immunodeficient (scid) mice, Ku8B-deficient mice are also defective for signal joint formation. Both hairpin coding ends and blunt full-length signal ends accumulate. Contrary to expectation, Ku86 is evidently not required for protection of either type of V(D)J recombination intermediate. Instead, V(D)J recombination appears to be arrested after the cleavage step in Ku86-deficient mice. We suggest that Ku86 may be required to remodel or disassemble DNA-protein complexes containing broken ends, making them available for further processing and joining.
Publisher
CELL PRESS
Issue Date
1996-08
Language
ENG
Keywords

STRAND-BREAK-REPAIR; DEPENDENT PROTEIN-KINASE; COMBINED IMMUNE-DEFICIENCY; DNA END-BINDING; CELL RECEPTOR-GAMMA; PRE-B CELLS; KU-AUTOANTIGEN; MAMMALIAN-CELLS; SCID MICE; MOUSE THYMOCYTES

Citation

CELL, v.86, no.3, pp.379 - 389

ISSN
0092-8674
DOI
10.1016/S0092-8674(00)80111-7
URI
http://hdl.handle.net/10203/74796
Appears in Collection
BS-Journal Papers(저널논문)
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