MODY Type 11

Definition

Heterozygous pathogenic variants in the BLK gene impair the regulatory role of B-lymphocyte kinase in insulin secretion in the beta cell; MODY11 is an extremely rare subtype accounting for less than 1% of all MODY cases. Some studies have noted that the evidence for BLK's pathogenicity as a MODY gene is weak.

Gene/region analyzed

BLK - Exon 1, BLK - Exon 2, BLK - Exon 3, BLK - Exon 4, BLK - Exon 5, BLK - Exon 6, BLK - Exon 7, BLK - Exon 8, BLK - Exon 9, BLK - Exon 10, BLK - Exon 11, BLK - Exon 12, BLK - Exon 13

Method

Sequence analysis

Accepted sample types

EDTA blood, AF, CVS

Inheritance

Hereditary, autosomal dominant.

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