Gene
SDHA
succinate dehydrogenase complex flavoprotein subunit A
- HGNC 10680↗(opens in a new tab)
- Ensembl gene ENSG00000073578↗(opens in a new tab)
- NCBI Gene 6389↗(opens in a new tab)
Summary
- Cytogenetic location
- 5p15.33
- Coordinates
- Chromosome 5: 218,320–257,082 (GRCh38)
- Exons
- 15 exons across 38.8 kb
- Protein
- 664 amino acids
- Inheritance
- Autosomal dominant
- Also known as
- FP, SDHF
- Previous symbols
- SDH2
- MANE Select transcript
- ENST00000264932.11NM_004168.4
- Locus type
- gene with protein product
- Very high
- Confident
- Low
- Very low
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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Get my Genome TestCurated reports relevant to this gene
Hereditary Pheochromocytoma and Paraganglioma
- Hereditary pheochromocytoma-paragangliomaAutosomal dominant
A group of syndromes (PPGL types 1-7, TMEM127- and MAX-related) in which variants in SDHB, SDHD, SDHC, SDHA, SDHAF2, MAX or TMEM127 raise the risk of pheochromocytoma and paraganglioma.
- ClinGen rates the link definitive
- 6 GenCC submitters rate it definitive or strong
What knowing changes
Regular blood or urine metanephrine testing and periodic whole-body MRI help find tumours early, before they cause blood-pressure crises; relatives can be offered testing.
Malignant Endocrine Neoplasm
A malignant neoplasm affecting the endocrine glands. Representative examples include thyroid gland carcinoma, parathyroid gland carcinoma, pituitary gland carcinoma, and adrenal cortex carcinoma.
- 5 GenCC submitters rate it definitive or strong
Urinary and Renal System Tumour
- Urinary System Neoplasm
A benign or malignant, primary or metastatic neoplasm involving the urinary system.
- 5 GenCC submitters rate it definitive or strong
Broader groupings
Leigh Syndrome
One of 69 genes linked to it
A progressive neurological disease defined by specific neuropathological features associating brainstem and basal ganglia lesions.
- ClinGen rates the link moderate
Inheritance
- Autosomal dominant
- One changed copy, from either parent, is enough. A parent who has one passes it on to each child with a one-in-two chance.
How SDHA is laid out
- Coding sequence
- Untranslated region
- Intron
- Direction of transcription
- Known disease-causing sites
- Stretches short reads find hard
Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.
Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).
Nearby genes from Ensembl release 116, on GRCh38.
Exons make up 2.7 kb of the gene's 38.8 kb; 1,995 of those bases code for the protein.
How a genome test reads SDHA
306
positions in SDHA where ClinVar classes a change as pathogenic or likely pathogenic
306 of them (100%) fall in regions short reads find hard.
- Low mappability (110 sites)Regions where 100 bp or 250 bp reads cannot be placed uniquely.
- Segmental duplications (306 sites)Copies of at least 1 kb sharing at least 90% identity with another part of the genome (paralogues, pseudogenes); reads may align to the wrong copy.
A whole genome covers all 38.8 kb of SDHA, introns included, not only the 2.7 kb of exons.
Genes linked to the same conditions
- SDHAF2succinate dehydrogenase complex assembly factor 2Shares 3 conditions
- RETret proto-oncogeneShares 2 conditions
- SDHBsuccinate dehydrogenase complex iron sulfur subunit BShares 2 conditions
- TP53tumor protein p53Shares 2 conditions
- AIFM1apoptosis inducing factor mitochondria associated 1Shares 1 condition
- ATP5MKATP synthase membrane subunit kShares 1 condition
- BAP1BRCA1 associated deubiquitinase 1Shares 1 condition
- BTDbiotinidaseShares 1 condition
- CDC73cell division cycle 73Shares 1 condition
- COX10cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10Shares 1 condition
- DLATdihydrolipoamide S-acetyltransferaseShares 1 condition
- DLDdihydrolipoamide dehydrogenaseShares 1 condition
Traits linked to SDHA
The Human Phenotype Ontology lists these as features of the rare conditions SDHA can cause. Each trait's page lists the other genes linked to it.
Abnormality of the nervous system
- Global developmental delay
- Headache
- Inability to coordinate movements when walking
- Instability or lack of coordination of central trunk muscles
- Loss of developmental milestones
- Unsteady walk
- Abnormal gait
- Ataxia
Show 34 more traits
- Babinski sign
- Balance impairment
- Brisk knee jerk
- Clumsiness
- Cranial nerve paralysis
- Degeneration of cerebellum
- Depression
- Dysesthesia
- Episodic paroxysmal anxiety
- Generalized myoclonic seizure
- Hyperactive deep tendon reflexes
- Limb ataxia
- Motor delay
- Progressive degeneration of movement
- Progressive psychomotor deterioration
- Recurrent paroxysmal headache
- Upper limb hyperreflexia
- Communication delay
- Cranial nerve compression
- Epilepsy
- Focal myoclonic seizure
- Inability to move vocal cords
- Irritability
- Moderate global developmental delay
- Panic attack
- Tremor
- Dementia
- Loss of ability to walk
- Cognitive impairment
- Dystonia
- Increased reflexes
- Jerking
- Leukoencephalopathy
- Progressive leukoencephalopathy
Abnormality of the cardiovascular system
- Disease of the heart muscle
- Enlarged heart
- Reduced left ventricular ejection fraction
- Stretched and thinned heart muscle
- Left ventricular systolic dysfunction
- Abnormal heart rate
- Abnormal left ventricular function
- Abnormality of the cardiovascular system
Show 21 more traits
- Cardiac failure
- Decrease in blood pressure upon standing up
- Elevated heart rate
- Enlarged and thickened heart muscle
- Fainting spell
- High blood pressure
- Hypertension associated with pheochromocytoma
- Hypertensive retinopathy
- Left ventricular hypertrophy
- Left ventricular noncompaction
- Palpitations
- Positive regitine blocking test
- Sinus tachycardia
- Swollen lymph nodes in center of chest
- Abnormal atrioventricular conduction
- Arachnoid hemangiomatosis
- Cardiogenic shock
- Hypertensive crisis
- Inflammation of heart muscle
- Noncompaction cardiomyopathy
- Swollen lymph nodes
External links
Gene records
Transcript
Protein
Where this comes from
- HGNC: the gene's symbol, name, other symbols and location.
- MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
- GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
- The ACMG secondary findings list, SF v3.2.
- ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
- The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
- Data release 2026.10.16-c6eea6bf.
Cite this page
Dante Labs. (n.d.). SDHA gene: succinate dehydrogenase complex flavoprotein subunit A. Retrieved 17 October 2026, from https://dantelabs.com/genes/sdha
Dante Labs. SDHA gene: succinate dehydrogenase complex flavoprotein subunit A [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/sdha
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