Annotation Detail

Information
Associated Genes
ELN
Associated Variants
GLB1 p.Tyr591Cys (p.Y591C) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Tyr591His (p.Y591H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Tyr591Asn (p.Y591N) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Arg482His (p.R482H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Arg201His (p.R201H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Arg59His (p.R59H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
ELN p.Gly678Asp (p.G678D) ( ENST00000252034.12, ENST00000320399.10, ENST00000320492.11, ENST00000357036.9, ENST00000380553.8, ENST00000380562.8, ENST00000380575.8, ENST00000380576.9, ENST00000380584.8, ENST00000414324.5, ENST00000429192.5, ENST00000445912.5, ENST00000458204.5, ENST00000621115.4, ENST00000692049.1 )
GLB1 p.Tyr591Cys (p.Y591C) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Tyr591His (p.Y591H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Tyr591Asn (p.Y591N) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Arg482His (p.R482H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Arg201His (p.R201H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
GLB1 p.Arg59His (p.R59H) ( ENST00000307363.10, ENST00000307377.12, ENST00000399402.7 )
ELN p.Gly678Asp (p.G678D) ( ENST00000252034.12, ENST00000320399.10, ENST00000320492.11, ENST00000357036.9, ENST00000380553.8, ENST00000380562.8, ENST00000380575.8, ENST00000380576.9, ENST00000380584.8, ENST00000414324.5, ENST00000429192.5, ENST00000445912.5, ENST00000458204.5, ENST00000621115.4, ENST00000692049.1 )
Associated Disease
congenital heart defects
Source Database
DisGeNET
Description
Interestingly, all patients with cardiac involvement were homozygous for one of these mutations: R59H, Y591C, Y591N, or IVS14-2A>G. In contrast, all other patients were compound heterozygous for one of the following mutations: R201H, R482H, G579D, IVS8+2T>C. Although we could not directly correlate the presence of cardiac abnormalities with specific genetic lesions, the mutations identified in patients with cardiomyopathy fell in the GLB1 cDNA region common to the lysosomal enzyme and the Hbeta-Gal-related protein, also known as the elastin binding protein (EBP).
Pubmed
10737981
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.00081432561624091
Year of publication
2000
Drugs