DES

Chr 2

desmin

Also known as: CDCD3, CSM1, CSM2, LGMD1D, LGMD1E, LGMD2R

This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMyopathy, myofibrillar, 1
UniProtCardiomyopathy, dilated, 1I
UniProtNeurogenic scapuloperoneal syndrome Kaeser type

Clinical highlights

Gene-disease validity (ClinGen)
arrhythmogenic right ventricular cardiomyopathy · ADModerateconsider for supplementary testing2 gene-disease associations curated in total
Interpreting a novel variant
This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Curated gene-level mechanism — a prior for triage, not a per-variant call.
12
Active trials
6725
Pubs (1 yr)
P/LP submissions
P/LP missense
0.60
LOEUF
Multiple*
Mechanism· predicted
📖
GeneReview available — DES
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.60LOEUF
pLI 0.009
Z-score 3.05
OE 0.33 (0.190.60)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
1.77Z-score
OE missense 0.71 (0.630.79)
206 obs / 291.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.33 (0.190.60)
00.351.4
Missense OE?0.71 (0.630.79)
00.61.4
Synonymous OE?0.87
01.21.6
LoF obs/exp: 8 / 24.2Missense obs/exp: 206 / 291.1Syn Z: 1.11

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DES · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Lymphogranuloma Venereumno Anorectal SymptomsDoxycycline

Multicentre Study Evaluating the Efficacy of a 7-day Course of Doxycycline for the Treatment of Asymptomatic Anal Lymphogranuloma Venereum.

NOT YET RECRUITING
NCT07767422Phase PHASE2University Hospital, BordeauxStarted 2026-09
Doxycline
Metastatic Colon AdenocarcinomaMetastatic Rectal AdenocarcinomaStage III Colon Cancer AJCC v8

Testing the Addition of Nivolumab to Standard Treatment for Patients With Metastatic or Unresectable Colorectal Cancer That Have a BRAF Mutation

ACTIVE NOT RECRUITING
NCT05308446Phase PHASE2National Cancer Institute (NCI)Started 2022-07-19
CetuximabEncorafenibNivolumab
Bilateral Vestibular HypofunctionBilateral Vestibular DeficiencyBilateral Vestibulopathy

Long Term Outcomes After Vestibular Implantation

RECRUITING
NCT06500975Phase NAJohns Hopkins UniversityStarted 2024-12-01
Labyrinth Devices MVI™ Multichannel Vestibular Implant System
X-Linked Retinitis Pigmentosa

Follow-up Gene Therapy Trial for the Treatment of X-linked Retinitis Pigmentosa Associated With Variants in the RPGR Gene

ACTIVE NOT RECRUITING
NCT04794101Phase PHASE3Janssen Research & Development, LLCStarted 2020-12-04
Genetic: AAV5-hRKp.RPGR Intermediate DoseGenetic: AAV5-hRKp.RPGR Low Dose
Anatomic Stage II Breast Cancer AJCC v8Anatomic Stage IIA Breast Cancer AJCC v8Anatomic Stage IIB Breast Cancer AJCC v8

CompassHER2-pCR: Decreasing Chemotherapy for Breast Cancer Patients After Pre-surgery Chemo and Targeted Therapy

ACTIVE NOT RECRUITING
NCT04266249Phase PHASE2ECOG-ACRIN Cancer Research GroupStarted 2020-03-13
DocetaxelLumpectomyMastectomy
Prostate Cancer

CHLORDECONE EXPOSURE AND PROSTATE CANCER IN THE FRENCH REGION OF MARTINIIQUE

RECRUITING
NCT06773806Institut National de la Santé Et de la Recherche Médicale, FranceStarted 2026-04-15
Malignant Solid Neoplasm

Immune Checkpoint Inhibitor Toxicity Risk Prediction in Solid Tumors

RECRUITING
NCT04871542SWOG Cancer Research NetworkStarted 2021-09-13
Biospecimen CollectionQuestionnaire Administration
Intracranial MeningiomaRecurrent MeningiomaNF2 Gene Mutation

Vismodegib, FAK Inhibitor GSK2256098, Capivasertib, and Abemaciclib in Treating Patients With Progressive Meningiomas

RECRUITING
NCT02523014Phase PHASE2Alliance for Clinical Trials in OncologyStarted 2015-09-28
VismodegibFAK Inhibitor GSK2256098Capivasertib
Diffuse Large B-Cell LymphomaDiffuse Large B-Cell Lymphoma, Not Otherwise SpecifiedDouble-Expressor Lymphoma

Testing the Addition of a New Anti-cancer Drug, Venetoclax, to Usual Chemotherapy for High Grade B-cell Lymphomas

ACTIVE NOT RECRUITING
NCT03984448Phase PHASE2, PHASE3National Cancer Institute (NCI)Started 2019-10-22
Biospecimen CollectionBone Marrow BiopsyComputed Tomography
Non-syndromic Retinitis Pigmentosa

Dose-escalation Study to Evaluate the Safety and Tolerability of GS030 in Subjects With Retinitis Pigmentosa

ACTIVE NOT RECRUITING
NCT03326336Phase PHASE1, PHASE2GenSight BiologicsStarted 2018-09-26
Gene therapy: GS030-DP AND Medical device: GS030-MD
Melanoma (Skin)

High-Dose Interferon Alfa in Treating Patients With Stage II or Stage III Melanoma

ACTIVE NOT RECRUITING
NCT00003641Phase PHASE3ECOG-ACRIN Cancer Research GroupStarted 1999-03-25
interferon alfa-2bobservation
Hepatitis C VirusMutation AbnormalityRNA Positive

Genotype and Subtype Mapping of the Hepatitis C Virus

NOT YET RECRUITING
NCT07503288Assistance Publique - Hôpitaux de ParisStarted 2026-06-01