SOD1

Chr 21

superoxide dismutase 1

Also known as: ALS, ALS1, HEL-S-44, IPOA, SOD, STAHP, hSod1, homodimer

The protein encoded by this gene binds copper and zinc ions and is one of two isozymes responsible for destroying free superoxide radicals in the body. The encoded isozyme is a soluble cytoplasmic protein, acting as a homodimer to convert naturally-occuring but harmful superoxide radicals to molecular oxygen and hydrogen peroxide. The other isozyme is a mitochondrial protein. In addition, this protein contains an antimicrobial peptide that displays antibacterial, antifungal, and anti-MRSA activity against E. coli, E. faecalis, S. aureus, S. aureus MRSA LPV+, S. agalactiae, and yeast C. krusei. Mutations in this gene have been implicated as causes of familial amyotrophic lateral sclerosis. Rare transcript variants have been reported for this gene. [provided by RefSeq, Jul 2020]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtAmyotrophic lateral sclerosis 1
UniProtSpastic tetraplegia and axial hypotonia, progressive

Clinical highlights

Gene-disease validity (ClinGen)
amyotrophic lateral sclerosis type 1 · ADDefinitivesufficient evidence for diagnostic panels
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.Based on population constraint only.
12
Active trials
1245
Pubs (1 yr)
P/LP submissions
P/LP missense
0.98
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — SOD1
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.98LOEUF
pLI 0.177
Z-score 1.62
OE 0.31 (0.130.98)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.84Z-score
OE missense 0.75 (0.610.92)
65 obs / 87.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.31 (0.130.98)
00.351.4
Missense OE?0.75 (0.610.92)
00.61.4
Synonymous OE?1.28
01.21.6
LoF obs/exp: 2 / 6.4Missense obs/exp: 65 / 87.0Syn Z: -1.27

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SOD1 · 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

Rheumatoid ArthritisOsteoarthritisSarcopenia

Effect of Exercise Type on Muscle Quality in Patients With OA, SARC and RA: an Explorative Study

NOT YET RECRUITING
NCT06480643Phase NAAmsterdam UMC, location VUmcStarted 2024-12-01
High load exercise typeLow load exercise type
Amyotrophic Lateral Sclerosis

A Study to Learn More About the Long-Term Safety of Tofersen (Qalsody) in Chinese Participants With SOD-1 Amyotrophic Lateral Sclerosis (ALS)

ACTIVE NOT RECRUITING
NCT07223723Phase PHASE4BiogenStarted 2025-12-11
Tofersen
Amyotrophic Lateral Sclerosis

A Study to Learn More About the Long-Term Safety of Tofersen (Qalsody) in Participants With Superoxide Dismutase 1 (SOD-1) Amyotrophic Lateral Sclerosis (ALS)

RECRUITING
NCT07259980BiogenStarted 2026-01-02
Tofersen
Physiological AdaptationsSport PerformanceAerobic Capacity

Molecular Hydrogen Inhalation: Effects on Health, Exercise Capacity and Inflammatory Response - in Vivo/in Vitro Studies

NOT YET RECRUITING
NCT07130942Phase NAGdansk University of Physical Education and SportStarted 2025-10-01
Inhalation using a molecular hydrogen generator
Amyotrophic Lateral Sclerosis

A Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of RAG-17 in Subjects With Amyotrophic Lateral Sclerosis (ALS) With Superoxide Dismutase Type 1 (SOD1) Gene Mutation

RECRUITING
NCT06556394Phase PHASE1Ractigen Therapeutics.Started 2024-12-24
RAG-17Placebo
Amyotrophic Lateral Sclerosis

Longitudinal Assessment of Autonomic and Sensory Nervous System in ALS

RECRUITING
NCT05747937Phase NAIstituti Clinici Scientifici Maugeri SpAStarted 2021-05-15
Skin biopsyCardiovascular Reflexes testingAdministration of clinical scales evaluating autonomic symptoms, pain small fiber neuropathy symptoms
B Acute Lymphoblastic LeukemiaB Acute Lymphoblastic Leukemia With t(9;22)(q34.1;q11.2); BCR-ABL1B Acute Lymphoblastic Leukemia, BCR-ABL1-Like

Studying the Effect of Levocarnitine in Protecting the Liver From Chemotherapy for Leukemia or Lymphoma

ACTIVE NOT RECRUITING
NCT05602194Phase PHASE3Children's Oncology GroupStarted 2023-08-24
Biospecimen CollectionCalaspargase PegolLevocarnitine
Transgender Individuals

Effects of Gender-Affirming Hormone Therapy on Cardiovascular, Metabolic, and Mental Health Outcomes in Transgender Adults.

NOT YET RECRUITING
NCT07394400Milagros Rocha BarajasStarted 2026-02-14
Inflammatory Bowel Diseases

Markers of Oxidative Stress in Inflammatory Bowel Diseases: Risk Factors and Implications for a Dietetic Approach

ACTIVE NOT RECRUITING
NCT04513015Phase NAUniversità Politecnica delle MarcheStarted 2019-12-15
Antioxidant dietNormal dietetic scheme
Neurodegenerative DiseaseBehavioral Variant Frontotemporal Dementia (bvFTD)Primary Progressive Aphasia(PPA)

Tracking and Predicting How Brain Damage Spreads in Neurodegenerative Diseases

ENROLLING BY INVITATION
NCT07567664Phase NAIRCCS San RaffaeleStarted 2017-06-01
3 Tesla MRI without contrast mediumBlood sample for genetic analysisCerebrospinal fluid sampling (CSF)
Amyotrophic Lateral Sclerosis

A Study to Investigate the Safety and Pharmacodynamics of a Single Intrathecal Injection (IT) of INS1202 in Participants With Amyotrophic Lateral Sclerosis (ALS)

RECRUITING
NCT07290062Phase PHASE1Insmed Gene Therapy LLCStarted 2026-01-09
INS1202
Amyotrophic Lateral Sclerosis Associated With a SOD1 Gene Mutation

A Study of BIIB067 (Tofersen) Initiated in Clinically Presymptomatic Adults With a Confirmed Superoxide Dismutase 1 Mutation

ACTIVE NOT RECRUITING
NCT04856982Phase PHASE3BiogenStarted 2021-05-17
TofersenPlacebo