Annotation Detail

Information
Associated Genes
MECP2
Associated Variants
MECP2 p.Arg282Ter (p.R282*) ( ENST00000407218.5, ENST00000453960.7, ENST00000303391.11, ENST00000415944.4, ENST00000628176.2 )
MECP2 p.Arg282Gly (p.R282G) ( ENST00000303391.11, ENST00000407218.5, ENST00000415944.4, ENST00000453960.7, ENST00000628176.2 )
MECP2 p.Arg282Ter (p.R282*) ( ENST00000303391.11, ENST00000407218.5, ENST00000415944.4, ENST00000453960.7, ENST00000628176.2 )
MECP2 p.Arg282Gly (p.R282G) ( ENST00000303391.11, ENST00000407218.5, ENST00000415944.4, ENST00000453960.7, ENST00000628176.2 )
Associated Disease
Rett syndrome
Source Database
DisGeNET
Description
Among 60 patients 57 girls with a clinical picture of RTT had normal female karyotype (46,XX), one boy had normal male karyotype in peripheral lymphocytes (46,XY) and two boys had a mosaic form of Kleinfelter's syndrome (47,XXY/46,XY) in peripheral lymphocytes or muscle cells (with MeCP2 mutation R270X).
Pubmed
11738872
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.8
Year of publication
2001
Drugs