Maturity-onset Diabetes Of The Young, Type 2; Mody2

Description

MODY is a form of NIDDM (OMIM ) characterized by monogenic autosomal dominant transmission and early age of onset. For a general phenotypic description and a discussion of genetic heterogeneity of MODY, see {606391}.In a review of the various forms of MODY, Fajans et al. (2001) stated that glucokinase-related MODY2 is a common form of the disorder, especially in children with mild hyperglycemia and in women with gestational diabetes and a family history of diabetes. It has been described in persons of all racial and ethnic groups. More than 130 MODY-associated mutations have been found in the glucokinase gene. Heterozygous mutations in glucokinase are associated with a mild form of nonprogressive hyperglycemia that is usually asymptomatic at diagnosis and is treated with diet alone. The mild fasting hyperglycemia with blood glucose concentrations of 110 to 145 mg/deciliter and impaired glucose tolerance in most affected carriers may be recognized by biochemical testing at a young age, possibly as early as birth. About 50% of the women who are carriers may have gestational diabetes. Less than 50% of the carriers have overt diabetes; many of those who do are obese or elderly. Two percent of MODY2 patients require insulin therapy. Diabetes-associated complications are rare in this form of MODY. MODY was found in 13% of the Caucasian NIDDM families collected in France by Froguel et al. (1991). Gidh-Jain et al. (1993) found that GCK mutations accounted for 56% of MODY families in France.

Clinical Features

Phenotypes and symptoms related to Maturity-onset Diabetes Of The Young, Type 2; Mody2

  • Type II diabetes mellitus
  • Hyperglycemia
  • Glucose intolerance
  • Maternal diabetes
  • Maturity-onset diabetes of the young
  • Transient neonatal diabetes mellitus

Incidence and onset information

— Currently we don't have prevalence information about this disease (Not enough data available about incidence and published cases.)
No data available about the known clinical features onset.

Alternative names

Maturity-onset Diabetes Of The Young, Type 2; Mody2 Is also known as mody, glucokinase-related, mody, type 2.

Researches and researchers

Currently, we don't have any information about doctors, researches or researchers related to this disease. Please contact us if you would like to appear here.


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Maturity-onset Diabetes Of The Young, Type 2; Mody2 Recommended genes panels

Panel Name, Specifity and genes Tested/covered
MitoMetĀ®Plus aCGH Analysis.

By Baylor Miraca Genetics Laboratories (United States).

RGS9, RHO, GRK1, RLBP1, RNASEL, BCS1L, RP1, RP2, RP9, RPE65, RPGR, RPL35A, MRPL3, RPS14, RS1, SAG, SARDH, SCO2, SCP2, SDHB , (...)

View the complete list with 612 more genes
Specificity
1 %
Genes
50 %
Congenital Hyperinsulinism Evaluation.

By Athena Diagnostics Inc (United States).

GCK, GLUD1, ABCC8, KCNJ11
Specificity
50 %
Genes
100 %
Neonatal Diabetes Mellitus Evaluation.

By Athena Diagnostics Inc (United States).

GCK, ABCC8, INS, PDX1, KCNJ11
Specificity
40 %
Genes
100 %
Monogenic Diabetes (MODY) Five Gene Evaluation (GCK,HNF1A,HNF1B,HNF4A,IPF1).

By Athena Diagnostics Inc (United States).

HNF1A, HNF1B, GCK, HNF4A, PDX1
Specificity
20 %
Genes
50 %
GCK (NDM) DNA Sequencing Test.

By Athena Diagnostics Inc (United States).

GCK
Specificity
100 %
Genes
50 %
GCK (CH) DNA Sequencing Test.

By Athena Diagnostics Inc (United States).

GCK
Specificity
100 %
Genes
50 %
GCK (MODY2) DNA Sequencing and Deletion Test.

By Athena Diagnostics Inc (United States).

GCK
Specificity
100 %
Genes
50 %
Monogenic Diabetes (MODY) Four Gene Evaluation (GCK,HNF1A, HNF1B, HNF4A).

By Athena Diagnostics Inc (United States).

HNF1A, HNF1B, GCK, HNF4A
Specificity
25 %
Genes
50 %

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Sources and references

You can check the following sources for additional information.

OMIM Rare Disease Search Engine

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