Table 2.

Different types of MODY diabetes including the affected gene focus, its localisation, and frequency.

Type of MODY	Affected gene	Gene localisation	Frequency
MODY 1	HNF4A (hepatocyte nuclear factor-4-alpha), hepatic transcription factor	Chromosome 20 q13.12	5% of cases
MODY 2: chromosome	Glucokinase	7 p13	10–15% of cases, but the commonest subtype of monogenic diabetes in the paediatric diabetes clinic (27)
MODY 3	HNF1A (hepatocyte nuclear factor-1-alpha), hepatic transcription factor	Chromosome 12 q24.2;	With 65% of cases, the most common form of MODY in Europe that results in familial symptomatic diabetes; manifestation occurs in early puberty
MODY 4	PDX1 (pancreas/duodenum homeobox protein-1)	Chromosome 13 q12.1	N/a
MODY 5	TCF2 (hepatic transcription factor-2), hepatic transcription factor	Chromosome 17 cen-q21.3	N/a
MODY 6	NEUROD1 (neurogenic differentiation 1)	Chromosome 2 q32	N/a
MODY 7	KLF11 (Krueppel-like factor 11)	Chromosome 2 p25	N/a
MODY 8: chromosome 9 q34	CEL (carboxyl ester lipase)	Chromosome 9 q34	N/a
MODY 9	PAX4 (paired box gene 4)	Chromosome 7 q32	N/a
MODY 10	INS (insulin gene)	Chromosome 11 p15.5	N/a
MODY 11	BLK (B-lymphoid tyrosine kinase)	Chromosome 8 p23	N/a
PNDM	Permanent neonatal diabetes mellitus; activating mutations in ABCC8 or KCNJ11 gene	Chromosome 11	N/a
TNDM	Transient neonatal diabetes mellitus	Among others due to imprinting defects on chromosome 6	N/a