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2014 ; 54
(5
): 791-804
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The 3M complex maintains microtubule and genome integrity
#MMPMID24793695
Yan J
; Yan F
; Li Z
; Sinnott B
; Cappell KM
; Yu Y
; Mo J
; Duncan JA
; Chen X
; Cormier-Daire V
; Whitehurst AW
; Xiong Y
Mol Cell
2014[Jun]; 54
(5
): 791-804
PMID24793695
show ga
CUL7, OBSL1, and CCDC8 genes are mutated in a mutually exclusive manner in 3M and
other growth retardation syndromes. The mechanism underlying the function of the
three 3M genes in development is not known. We found that OBSL1 and CCDC8 form a
complex with CUL7 and regulate the level and centrosomal localization of CUL7,
respectively. CUL7 depletion results in altered microtubule dynamics,
prometaphase arrest, tetraploidy, and mitotic cell death. These defects are
recaptured in CUL7 mutated 3M cells and can be rescued by wild-type, but not by
3M patient-derived CUL7 mutants. Depletion of either OBSL1 or CCDC8 results in
defects and sensitizes cells to microtubule damage similarly to loss of CUL7
function. Microtubule damage reduces the level of CCDC8 that is required for the
centrosomal localization of CUL7. We propose that CUL7, OBSL1, and CCDC8 proteins
form a 3M complex that functions in maintaining microtubule and genome integrity
and normal development.
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