PMID- 10373536
OWN - NLM
STAT- MEDLINE
DCOM- 19990722
LR  - 20190508
IS  - 0270-7306 (Print)
IS  - 0270-7306 (Linking)
VI  - 19
IP  - 7
DP  - 1999 Jul
TI  - Fanconi anemia proteins FANCA, FANCC, and FANCG/XRCC9 interact in a functional
      nuclear complex.
PG  - 4866-73
AB  - Fanconi anemia (FA) is an autosomal recessive cancer susceptibility syndrome with
      at least eight complementation groups (A to H). Three FA genes, corresponding to 
      complementation groups A, C, and G, have been cloned, but their cellular function
      remains unknown. We have previously demonstrated that the FANCA and FANCC
      proteins interact and form a nuclear complex in normal cells, suggesting that the
      proteins cooperate in a nuclear function. In this report, we demonstrate that the
      recently cloned FANCG/XRCC9 protein is required for binding of the FANCA and
      FANCC proteins. Moreover, the FANCG protein is a component of a nuclear protein
      complex containing FANCA and FANCC. The amino-terminal region of the FANCA
      protein is required for FANCG binding, FANCC binding, nuclear localization, and
      functional activity of the complex. Our results demonstrate that the three cloned
      FA proteins cooperate in a large multisubunit complex. Disruption of this complex
      results in the specific cellular and clinical phenotype common to most FA
      complementation groups.
FAU - Garcia-Higuera, I
AU  - Garcia-Higuera I
AD  - Department of Pediatric Oncology, Dana-Farber Cancer Institute, Department of
      Pediatrics, Children's Hospital, Harvard Medical School, Boston, Massachusetts,
      USA.
FAU - Kuang, Y
AU  - Kuang Y
FAU - Naf, D
AU  - Naf D
FAU - Wasik, J
AU  - Wasik J
FAU - D'Andrea, A D
AU  - D'Andrea AD
LA  - eng
GR  - P01 CA039542/CA/NCI NIH HHS/United States
GR  - P01-CA39542/CA/NCI NIH HHS/United States
GR  - R01-HL5725/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Mol Cell Biol
JT  - Molecular and cellular biology
JID - 8109087
RN  - 0 (Cell Cycle Proteins)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (FANCC protein, human)
RN  - 0 (FANCG protein, human)
RN  - 0 (Fanconi Anemia Complementation Group C Protein)
RN  - 0 (Fanconi Anemia Complementation Group G Protein)
RN  - 0 (Fanconi Anemia Complementation Group Proteins)
RN  - 0 (Nuclear Proteins)
RN  - 0 (Proteins)
SB  - IM
MH  - Animals
MH  - *Cell Cycle Proteins
MH  - Cell Line, Transformed
MH  - Cell Nucleus/metabolism
MH  - DNA-Binding Proteins/genetics/*metabolism
MH  - Fanconi Anemia/*metabolism
MH  - Fanconi Anemia Complementation Group C Protein
MH  - Fanconi Anemia Complementation Group G Protein
MH  - Fanconi Anemia Complementation Group Proteins
MH  - Humans
MH  - *Nuclear Proteins
MH  - Proteins/genetics/*metabolism
MH  - Rabbits
PMC - PMC84285
EDAT- 1999/06/22 00:00
MHDA- 1999/06/22 00:01
CRDT- 1999/06/22 00:00
PHST- 1999/06/22 00:00 [pubmed]
PHST- 1999/06/22 00:01 [medline]
PHST- 1999/06/22 00:00 [entrez]
AID - 10.1128/mcb.19.7.4866 [doi]
PST - ppublish
SO  - Mol Cell Biol. 1999 Jul;19(7):4866-73. doi: 10.1128/mcb.19.7.4866.