hyperaldosteronism, familial, type IV

disease
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Also known as familial hyperaldosteronism type IVHALD4hyperaldosteronism, familial, type IV

Summary

hyperaldosteronism, familial, type IV (MONDO:0014875) is a disease caused by CACNA1H (GenCC Strong), with 6 cohort genes.

At a glance

  • Prevalence: <1 / 1 000 000 (Worldwide) [Orphanet-validated]
  • Causal gene: CACNA1H (GenCC Strong)
  • Cohort genes: 6
  • ClinVar variants: 3,664

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

TypeClassValueGeographyValidation
Cases/families17WorldwideValidated
Point prevalence<1 / 1 000 000WorldwideValidated

Identifiers

Disease identifiers

FieldValue
Canonical namehyperaldosteronism, familial, type IV
Mondo IDMONDO:0014875
OMIM617027
Orphanet642671
DOIDDOID:0061250
UMLSC4310756
MedGen934723
GARD0025028
Is cancer (heuristic)no

Also known as: familial hyperaldosteronism type IV · HALD4 · hyperaldosteronism, familial, type IV · hyperaldosteronism, familial, type IV; HALD4

Data availability: 3,664 ClinVar variants · 1 GenCC gene-disease record.

Disease family

Classification path: disease › human disease › disease by body system or component › endocrine system disorderadrenal gland disorderadrenal cortex disorderadrenal gland hyperfunctionhyperaldosteronismprimary aldosteronismfamilial hyperaldosteronismhyperaldosteronism, familial, type IV

Related subtypes (4): glucocorticoid-remediable aldosteronism, familial hyperaldosteronism type II, familial hyperaldosteronism type III, aldosterone-producing adenoma with seizures and neurological abnormalities

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

ClinVar germline variants

600 retrieved; paginated sample, class counts are floors:

237 likely benign, 210 uncertain significance, 106 conflicting classifications of pathogenicity, 33 benign, 14 benign/likely benign

ClinVarVariant (HGVS)GeneClassificationReview
1000691NM_021098.3(CACNA1H):c.4814C>A (p.Pro1605His)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1001887NM_021098.3(CACNA1H):c.2231G>A (p.Arg744Gln)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1004965NM_021098.3(CACNA1H):c.3758G>A (p.Arg1253His)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1005249NM_021098.3(CACNA1H):c.1673C>T (p.Ser558Leu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1008653NM_021098.3(CACNA1H):c.3884T>C (p.Met1295Thr)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1011552NM_021098.3(CACNA1H):c.391G>A (p.Glu131Lys)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1011782NM_021098.3(CACNA1H):c.4823G>A (p.Arg1608His)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1013845NM_021098.3(CACNA1H):c.560C>T (p.Ser187Leu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1015188NM_021098.3(CACNA1H):c.3584G>A (p.Arg1195Gln)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1015270NM_021098.3(CACNA1H):c.6398G>T (p.Arg2133Leu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1015641NM_021098.3(CACNA1H):c.1691G>A (p.Arg564His)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1016006NM_021098.3(CACNA1H):c.3347G>A (p.Gly1116Glu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1020407NM_021098.3(CACNA1H):c.3143T>C (p.Leu1048Pro)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1021653NM_021098.3(CACNA1H):c.2995A>G (p.Met999Val)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1023131NM_021098.3(CACNA1H):c.3614G>C (p.Arg1205Pro)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1023368NM_021098.3(CACNA1H):c.6563G>A (p.Ser2188Asn)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1025264NM_021098.3(CACNA1H):c.3467G>A (p.Arg1156His)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1032113NM_021098.3(CACNA1H):c.2789+16C>TCACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1032116NM_021098.3(CACNA1H):c.974C>T (p.Pro325Leu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1038757NM_021098.3(CACNA1H):c.1046C>T (p.Ser349Leu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1041003NM_021098.3(CACNA1H):c.6281C>T (p.Ser2094Leu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1042192NM_021098.3(CACNA1H):c.2209G>A (p.Gly737Ser)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1044087NM_021098.3(CACNA1H):c.2545C>T (p.Pro849Ser)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1044825NM_021098.3(CACNA1H):c.1835A>C (p.Asn612Thr)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1046542NM_021098.3(CACNA1H):c.4741C>G (p.Leu1581Val)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1051387NM_021098.3(CACNA1H):c.1000C>T (p.Arg334Cys)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1051672NM_021098.3(CACNA1H):c.919G>A (p.Gly307Ser)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1052203NM_021098.3(CACNA1H):c.5957C>T (p.Ser1986Leu)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1054433NM_021098.3(CACNA1H):c.3359C>G (p.Pro1120Arg)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications
1057350NM_021098.3(CACNA1H):c.1765G>A (p.Ala589Thr)CACNA1HConflicting classifications of pathogenicitycriteria provided, conflicting classifications

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 4 · Orphanet: 6 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

GenCC gene–disease validity (cohort genes)

the Disease column is the GenCC-asserted condition — a cohort gene’s strongest validity may be for a related predisposition syndrome.

GeneClassificationInheritanceDiseaseRecords
CACNA1HStrongAutosomal dominanthyperaldosteronism, familial, type IV4

Orphanet rare-disease linkage (cohort genes)

GeneOrphanet IDRare disease
CACNA1HOrphanet:642671Familial hyperaldosteronism type IV
CACNA1HOrphanet:64280Childhood absence epilepsy
ABCA3Orphanet:2032Idiopathic pulmonary fibrosis
ABCA3Orphanet:217563Neonatal acute respiratory distress syndrome
ABCA3Orphanet:440402Interstitial lung disease due to ABCA3 deficiency
ABCA3Orphanet:685082Pediatric acute respiratory distress syndrome

Cohort genes → proteins

6 cohort genes, 6 distinct canonical proteins.

Evidence partition

SubsetGenes
multi_evidence6

Cohort genes (full)

SymbolHGNCEnsemblUniProtNameEvidence
CACNA1HHGNC:1395ENSG00000196557O95180Voltage-dependent T-type calcium channel subunit alpha-1Hgencc,clinvar
ANTKMTHGNC:14152ENSG00000103254Q9BQD7Adenine nucleotide translocase lysine N-methyltransferaseclinvar
RAB11FIP3HGNC:17224ENSG00000090565O75154Rab11 family-interacting protein 3clinvar
CHTF18HGNC:18435ENSG00000127586Q8WVB6Chromosome transmission fidelity protein 18 homologclinvar
C1QTNF8HGNC:31374ENSG00000184471P60827Complement C1q tumor necrosis factor-related protein 8clinvar
ABCA3HGNC:33ENSG00000167972Q99758Phospholipid-transporting ATPase ABCA3clinvar

Cohort function summary

Lead sentence per gene, UniProt-curated.

SymbolProtein nameFunction (lead sentence)
CACNA1HVoltage-dependent T-type calcium channel subunit alpha-1HVoltage-sensitive calcium channel that gives rise to T-type calcium currents.
ANTKMTAdenine nucleotide translocase lysine N-methyltransferaseMitochondrial protein-lysine N-methyltransferase that trimethylates adenine nucleotide translocases ANT2/SLC25A5 and ANT3/SLC25A6, thereby regulating mitochondrial respiration.
RAB11FIP3Rab11 family-interacting protein 3Downstream effector molecule for Rab11 GTPase which is involved in endocytic trafficking, cytokinesis and intracellular ciliogenesis by participating in membrane delivery.
CHTF18Chromosome transmission fidelity protein 18 homologChromosome cohesion factor involved in sister chromatid cohesion and fidelity of chromosome transmission.
C1QTNF8Complement C1q tumor necrosis factor-related protein 8May play a role as ligand of RXFP1.
ABCA3Phospholipid-transporting ATPase ABCA3Catalyzes the ATP-dependent transport of phospholipids such as phosphatidylcholine and phosphoglycerol from the cytoplasm into the lumen side of lamellar bodies, in turn participates in the lamellar bodies biogenesis and homeostasis of pul…

Protein-family classification

Druggable: 2 · Difficult: 0 · Unknown: 4 · Druggable fraction: 0.33

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

FamilyGenesFoldFDR
Ion channel118.6×0.112
Transporter113.0×0.112
Other/Unknown41.2×0.458

Per-gene assignment

SymbolFamilyDruggable?ECInterPro (top 3)
CACNA1HIon channelyesVDCC_T_a1, Ion_trans_dom, Volt_channel_dom_sf
ANTKMTOther/UnknownnoFAM173A/B, SAM-dependent_MTases_sf
RAB11FIP3Other/UnknownnoEF_hand_dom, EF-hand-dom_pair, Rab-bd_FIP-RBD
CHTF18Other/UnknownnoAAA+_ATPase, ATPase_AAA_core, P-loop_NTPase
C1QTNF8Other/UnknownnoC1q_dom, Collagen, Tumour_necrosis_fac-like_dom
ABCA3TransporteryesABC_transporter-like_ATP-bd, AAA+_ATPase, ABC2_TM

Expression context

Cohort genes with no expression data: 0.

4 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

BucketGenes
narrow (1-5 tissues)0
moderate (6-20)0
broad (>20)6
unknown0

Top tissues across cohort

TissueCohort genes
mucosa of transverse colon2
lower esophagus1
lower esophagus muscularis layer1
muscle layer of sigmoid colon1
anterior cingulate cortex1
cingulate cortex1
kidney epithelium1
pancreatic ductal cell1
right hemisphere of cerebellum1
left testis1
right testis1
ascending aorta1
right uterine tube1
thoracic aorta1
lower lobe of lung1
upper lobe of left lung1
upper lobe of lung1

Per-gene tissue summary (top 30)

SymbolBgee breadthFANTOM5 breadthSCXATop tissues
CACNA1H166broadmarkerlower esophagus muscularis layer, muscle layer of sigmoid colon, lower esophagus
ANTKMT243ubiquitousmarkeranterior cingulate cortex, cingulate cortex, mucosa of transverse colon
RAB11FIP3246ubiquitousmarkerkidney epithelium, pancreatic ductal cell, right hemisphere of cerebellum
CHTF18188ubiquitousyesright testis, left testis, mucosa of transverse colon
C1QTNF851yesright uterine tube, ascending aorta, thoracic aorta
ABCA3222ubiquitousmarkerlower lobe of lung, upper lobe of lung, upper lobe of left lung

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

SymbolInteractor count
CHTF181,878
CACNA1H1,564
ABCA31,436
ANTKMT1,032
RAB11FIP3863
C1QTNF8265

Structural data

PDB: 4 · AlphaFold-only: 2 · No structure: 0

Cohort genes with PDB structures (top 30)

SymbolUniProtPDB entries
CHTF18Q8WVB610
CACNA1HO951805
RAB11FIP3O751545
ABCA3Q997582

AlphaFold-only cohort genes (top 30 by pLDDT)

SymbolUniProtpLDDT
ANTKMTQ9BQD782.01
C1QTNF8P6082778.89

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 24. Enrichment computed across 6 evidence-associated genes (4 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 4 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

PathwayCohort genesFoldFDRSample cohort genes
Defective ABCA3 causes SMDP312855.0×0.004ABCA3
Diseases associated with surfactant metabolism1713.8×0.011ABCA3
Mechanical load activates signaling by PIEZO1 and integrins in osteocytes1167.9×0.028CACNA1H
ABC transporters in lipid homeostasis1150.3×0.028ABCA3
VxPx cargo-targeting to cilium1129.8×0.028RAB11FIP3
ABC transporter disorders1109.8×0.028ABCA3
Polymerase switching on the C-strand of the telomere1105.7×0.028CHTF18
Surfactant metabolism192.1×0.029ABCA3
NCAM signaling for neurite out-growth168.0×0.030CACNA1H
Smooth Muscle Contraction166.4×0.030CACNA1H
Cellular responses to mechanical stimuli164.9×0.030CACNA1H
NCAM1 interactions162.1×0.030CACNA1H
Disorders of transmembrane transporters134.8×0.049ABCA3
ABC-family protein mediated transport130.4×0.052ABCA3
Diseases of metabolism120.1×0.072ABCA3
Muscle contraction119.3×0.072CACNA1H
Axon guidance111.3×0.114CACNA1H
Nervous system development110.7×0.114CACNA1H
Cellular responses to stimuli17.9×0.145CACNA1H
Transport of small molecules16.3×0.171ABCA3
Developmental Biology13.6×0.272CACNA1H
Disease13.3×0.284ABCA3
Metabolism of proteins13.1×0.286ABCA3

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 6 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO termCohort genesFoldFDRSample cohort genes
positive regulation of protein homooligomerization12808.7×0.008ABCA3
regulation of phosphatidylcholine metabolic process11404.3×0.008ABCA3
protein localization to cleavage furrow11404.3×0.008RAB11FIP3
xenobiotic export from cell1936.2×0.008ABCA3
obsolete regulation of early endosome to recycling endosome transport1936.2×0.008RAB11FIP3
regulation of protein localization to centrosome1936.2×0.008RAB11FIP3
regulation of mitochondrial ATP synthesis coupled proton transport1936.2×0.008ANTKMT
negative regulation of adiponectin secretion1702.2×0.008RAB11FIP3
positive regulation of phospholipid efflux1702.2×0.008ABCA3
aldosterone biosynthetic process1561.7×0.008CACNA1H
obsolete early endosome to recycling endosome transport1561.7×0.008RAB11FIP3
peptidyl-lysine trimethylation1468.1×0.008ANTKMT
regulation of lipid biosynthetic process1468.1×0.008ABCA3
organelle assembly1468.1×0.008ABCA3
positive regulation of phospholipid transport1401.2×0.008ABCA3
cortisol biosynthetic process1351.1×0.008CACNA1H
positive regulation of DNA-directed DNA polymerase activity1351.1×0.008CHTF18
positive regulation of mitotic cytokinetic process1351.1×0.008RAB11FIP3
regulation of endocytic recycling1280.9×0.010RAB11FIP3
positive regulation of acrosome reaction1255.3×0.010CACNA1H
phosphatidylglycerol metabolic process1234.1×0.011ABCA3
regulation of vesicle-mediated transport1187.2×0.013RAB11FIP3
cellular response to potassium ion1175.5×0.013CACNA1H
phospholipid homeostasis1165.2×0.013ABCA3
xenobiotic transmembrane transport1156.0×0.013ABCA3
obsolete inorganic cation transmembrane transport1156.0×0.013CACNA1H
xenobiotic transport1140.4×0.013ABCA3
surfactant homeostasis1133.8×0.013ABCA3
phosphatidylcholine metabolic process1133.8×0.013ABCA3
calcium ion import1133.8×0.013CACNA1H

Therapeutics

Drug target analysis

Approved (phase 4): 1 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 5

Druggability breadth: 2 of 6 evidence-associated genes (33%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

SymbolExample approved molecule
CACNA1HPIMOZIDE

Top cohort targets by molecule count

SymbolMoleculesMax phase
CACNA1H104
ANTKMT00
RAB11FIP300
CHTF1800
C1QTNF800
ABCA300

Drugs targeting cohort genes (top 30)

MoleculeMax phaseTargets in cohort
PIMOZIDE4CACNA1H
MIBEFRADIL4CACNA1H
NIMODIPINE4CACNA1H
TACRINE4CACNA1H
CILNIDIPINE3CACNA1H
SUVECALTAMIDE2CACNA1H
FLUNARIZINE2CACNA1H
APINOCALTAMIDE2CACNA1H
Z1602CACNA1H
ULIXACALTAMIDE1CACNA1H

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 0.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

SymbolAssaysType breakdown
CACNA1H124Binding:102, Functional:17, ADMET:4, Toxicity:1
CHTF181Binding:1

Cohort genes with high screening signal

≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.

SymbolChEMBL assays
CACNA1H124

Pharmacogenomics

Cohort genes with a PharmGKB record: 6; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

10 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

CompoundMax phaseCohort target (bioactivity)
PIMOZIDE4CACNA1H
MIBEFRADIL4CACNA1H
NIMODIPINE4CACNA1H
TACRINE4CACNA1H
CILNIDIPINE3CACNA1H
SUVECALTAMIDE2CACNA1H
FLUNARIZINE2CACNA1H
APINOCALTAMIDE2CACNA1H
Z1602CACNA1H
ULIXACALTAMIDE1CACNA1H

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

TierDefinitionGenesSymbols
AApproved (phase 4 drug)1CACNA1H
BPhased (≥1) drug, not yet approved0
CDruggable family + PDB, no drug1ABCA3
DDruggable family + AlphaFold only, no drug0
EDifficult family or no structure, no drug4ANTKMT, RAB11FIP3, CHTF18, C1QTNF8

Undrugged target profiles

5 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

SymbolChEMBL assaysDrugged partners (top 3)
ANTKMT0
RAB11FIP30
CHTF181
C1QTNF80
ABCA30

Clinical trials & evidence

Clinical trials

Clinical trials: 0.