Annotation Detail

Information
Associated Genes
BSCL2
Associated Variants
HNRNPUL2-BSCL2 c.*506A>G, BSCL2 p.Asn152Ser (p.N152S) ( ENST00000405837.5, ENST00000683296.1, ENST00000403734.2, ENST00000278893.11, ENST00000407022.7, ENST00000684067.1, ENST00000449636.6, ENST00000684475.1, ENST00000682223.1, ENST00000679883.1, ENST00000360796.10, ENST00000421906.5, ENST00000403550.5, ENST00000524862.6 )
HNRNPUL2-BSCL2 c.*506A>G, BSCL2 p.Asn152Ser (p.N152S) ( ENST00000278893.11, ENST00000360796.10, ENST00000403550.5, ENST00000405837.5, ENST00000407022.7, ENST00000421906.5, ENST00000449636.6, ENST00000524862.6, ENST00000679883.1, ENST00000682223.1, ENST00000683296.1, ENST00000684067.1, ENST00000684475.1, ENST00000403734.2 )
Associated Disease
NEURONOPATHY, DISTAL HEREDITARY MOTOR, TYPE V
Source Database
DisGeNET
Description
Heterozygosity for mutations (N88S and P90L) in the N-glycosylation site of seipin/BSCL2 is associated with the autosomal dominant motor neuron diseases, spastic paraplegia 17 and distal hereditary motor neuropathy type V, referred to as 'seipinopathies'.
Pubmed
21750110
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.241628651232482
Year of publication
2011
Drugs