PMID- 8453669 OWN - NLM STAT- MEDLINE DCOM- 19930421 LR - 20220409 IS - 0092-8674 (Print) IS - 0092-8674 (Linking) VI - 72 IP - 5 DP - 1993 Mar 12 TI - A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. PG - 791-800 AB - Neurofibromatosis 2 (NF2) is a dominantly inherited disorder characterized by the occurrence of bilateral vestibular schwannomas and other central nervous system tumors including multiple meningiomas. Genetic linkage studies and investigations of both sporadic and familial tumors suggest that NF2 is caused by inactivation of a tumor suppressor gene in chromosome 22q12. We have identified a candidate gene for the NF2 tumor suppressor that has suffered nonoverlapping deletions in DNA from two independent NF2 families and alterations in meningiomas from two unrelated NF2 patients. The candidate gene encodes a 587 amino acid protein with striking similarity to several members of a family of proteins proposed to link cytoskeletal components with proteins in the cell membrane. The NF2 gene may therefore constitute a novel class of tumor suppressor gene. FAU - Trofatter, J A AU - Trofatter JA AD - Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown. FAU - MacCollin, M M AU - MacCollin MM FAU - Rutter, J L AU - Rutter JL FAU - Murrell, J R AU - Murrell JR FAU - Duyao, M P AU - Duyao MP FAU - Parry, D M AU - Parry DM FAU - Eldridge, R AU - Eldridge R FAU - Kley, N AU - Kley N FAU - Menon, A G AU - Menon AG FAU - Pulaski, K AU - Pulaski K AU - et al. LA - eng SI - GENBANK/L09219 SI - GENBANK/L09220 SI - GENBANK/L09221 SI - GENBANK/L09222 SI - GENBANK/L09223 SI - GENBANK/L09224 SI - GENBANK/L09225 SI - GENBANK/L10382 SI - GENBANK/L11353 SI - GENBANK/L33709 GR - HG00317/HG/NHGRI NIH HHS/United States GR - HG00672/HG/NHGRI NIH HHS/United States GR - NS24279/NS/NINDS NIH HHS/United States GR - etc. 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 - Cell JT - Cell JID - 0413066 RN - 0 (Blood Proteins) RN - 0 (Cytoskeletal Proteins) RN - 0 (Membrane Proteins) RN - 0 (Microfilament Proteins) RN - 0 (Phosphoproteins) RN - 0 (Proteins) RN - 0 (ezrin) RN - 144131-77-1 (moesin) RN - 144517-21-5 (radixin) SB - IM EIN - Cell. 1993 Nov 19;75(4):826. PMID: 8242753 MH - Amino Acid Sequence MH - Base Sequence MH - Blood Proteins/chemistry/genetics MH - Chromosome Aberrations MH - Chromosome Disorders MH - Chromosome Walking MH - *Cytoskeletal Proteins MH - *Genes, Tumor Suppressor MH - Humans MH - Membrane Proteins/chemistry/genetics MH - *Microfilament Proteins MH - Molecular Sequence Data MH - Neurofibromatosis 2/*genetics MH - Phosphoproteins/chemistry/genetics MH - Proteins/chemistry/genetics MH - Sequence Homology, Amino Acid EDAT- 1993/03/12 00:00 MHDA- 1993/03/12 00:01 CRDT- 1993/03/12 00:00 PHST- 1993/03/12 00:00 [pubmed] PHST- 1993/03/12 00:01 [medline] PHST- 1993/03/12 00:00 [entrez] AID - 0092-8674(93)90406-G [pii] AID - 10.1016/0092-8674(93)90406-g [doi] PST - ppublish SO - Cell. 1993 Mar 12;72(5):791-800. doi: 10.1016/0092-8674(93)90406-g.