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maturity-onset diabetes of the young type 10 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

maturity-onset diabetes of the young type 10

MONDO:0013240

Also known as:INS maturity-onset diabetes of the young (disease)MODY10maturity-onset diabetes of the young (disease) caused by mutation in INS

Overview

Any maturity-onset diabetes of the young in which the cause of the disease is a mutation in the INS gene.

GeneticsAutosomal dominant · INS (chr 11)

OnsetANTENATAL, INFANTILE

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include: Diabetes mellitus, Maturity-onset diabetes of the young, Hyperglycemia, and Intrauterine growth retardation and 1 more.

Organ System	Phenotype Count	Example Features
Hormones	2	Diabetes mellitus, Maturity-onset diabetes of the young

Genetic Landscape

Assembled from HGNC gene records, ClinGen gene-disease validity evidence, and ClinVar pathogenic variant data. Validity classifications reflect the strength of published evidence linking each gene to this disease.

INS encodes insulin (110 aa). Insulin decreases blood glucose concentration. It increases cell permeability to monosaccharides, amino acids and fatty acids. Highest expression in Pancreas (2,325 TPM) and Adrenal Gland (0.5 TPM).

Maturity-onset diabetes of the young type 10 is associated with mutations in the INS gene on chromosome 11.

INS is classified as a druggable target (Druggable Genome and Hormone Activity categories) with score 8.7.

Diagnosis

Drawn from GeneReviews diagnostic guidelines, newborn screening panel status (RUSP), and ClinGen gene-disease validity classifications. Reflects current clinical evidence and consensus where available.

Genetic testing for INS is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for maturity-onset diabetes of the young type 10 has been reported in the published literature.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for maturity-onset diabetes of the young type 10.

14 publications have been identified in PubMed for maturity-onset diabetes of the young type 10. Research spans Diagnostic / Biomarker (21%), Review / Meta-Analysis (21%), and Epidemiology / Natural History (21%).

Research Type	Count	% of Total
Testing and diagnosis research	3	21%

Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 4:38 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:613370

GARD

Genetic and Rare Diseases Info Center

GARD:15652

Questions for Your Care Team

→Should I pursue genetic testing for INS, and what would the results mean for my family?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

maturity-onset diabetes of the young type 10

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Research Landscape

Medical References

External Resources

OMIM

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

maturity-onset diabetes of the young type 10

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Research Landscape

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications