The role of noise and positive feedback in the onset of autosomal dominant diseases

BMC Systems Biology

Table 3 MODY characteristics

MODY type	Gene deficiency	Prevalence⁽¹⁾	Treatment^(1,2)	Simulated mean/std of onset age	Simulated mean/std insulin
1	Hnf-4α	Uncommon	Oral medication or insulin	10.3/1.2	0.18/0.02
2	glycokinase	Common	Diet and exercise	4.5/0.3	0.26/0.005
3	Hnf-1α	Most common	Oral medication or insulin	24.0/8.1	0.24/0.08
4	Pdx1	Rare	Oral medication or insulin	22.6/11.0	0.22/0.11
5	Hnf-1β	Rare	Insulin	9.3/0.4	0.08/0.004

Observed and simulated characteristics of the five major MODY types. The absolute magnitude of the simulated values is not as important as the relative values. The standard deviation in the age of onset is much larger for MODY3 and MODY4, which should be reflected in a range of onset ages in epidemiological data. Mean and standard deviation of the insulin levels is related to the severity of the disease. Data for "Prevalence" from [42, 44].

ISSN: 1752-0509