Familial temporal lobe epilepsy 8

disease
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Also known as epilepsy, familial temporal lobe, 8epilepsy, familial temporal lobe, type 8ETL8familial temporal lobe epilepsy type 8

Summary

Familial temporal lobe epilepsy 8 (MONDO:0014650) is a disease with 1 cohort gene.

At a glance

  • Cohort genes: 1
  • ClinVar variants: 56

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical namefamilial temporal lobe epilepsy 8
Mondo IDMONDO:0014650
OMIM616461
DOIDDOID:0060754
UMLSC4225318
MedGen909158
GARD0016116
Is cancer (heuristic)no

Also known as: epilepsy, familial temporal lobe, 8 · epilepsy, familial temporal lobe, type 8 · ETL8 · familial temporal lobe epilepsy type 8

Data availability: 56 ClinVar variants · 1 GenCC gene-disease record.

Disease family

Classification path: disease › human disease › disease by body system or component › nervous system disordercentral nervous system disorderbrain disorderepilepsyfocal epilepsyfamilial partial epilepsytemporal lobe epilepsyfamilial temporal lobe epilepsy 8

Related subtypes (5): familial temporal lobe epilepsy 2, familial temporal lobe epilepsy 4, familial temporal lobe epilepsy 7, epilepsy, familial temporal lobe, 1, familial mesial temporal lobe epilepsy

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

56 retrieved; paginated sample, class counts are floors:

37 uncertain significance, 13 likely benign, 5 benign, 1 pathogenic

ClinVarVariant (HGVS)GeneClassificationReview
372128NM_015973.5(GAL):c.116C>A (p.Ala39Glu)GALPathogenicno assertion criteria provided
1018689NM_015973.5(GAL):c.202C>T (p.Pro68Ser)GALUncertain significancecriteria provided, single submitter
1021546NM_015973.5(GAL):c.337G>A (p.Ala113Thr)GALUncertain significancecriteria provided, single submitter
1027299NM_015973.5(GAL):c.223+3G>CGALUncertain significancecriteria provided, single submitter
1037453NM_015973.5(GAL):c.7C>T (p.Arg3Ter)GALUncertain significancecriteria provided, single submitter
1037569NM_015973.5(GAL):c.13A>G (p.Ser5Gly)GALUncertain significancecriteria provided, multiple submitters, no conflicts
1039520NM_015973.5(GAL):c.200G>C (p.Arg67Pro)GALUncertain significancecriteria provided, single submitter
1043494NM_015973.5(GAL):c.263G>T (p.Arg88Leu)GALUncertain significancecriteria provided, single submitter
1051811NM_015973.5(GAL):c.216G>A (p.Met72Ile)GALUncertain significancecriteria provided, single submitter
1412001NC_000011.9:g.(?68453042)(68453136_?)delGALUncertain significancecriteria provided, single submitter
475911NM_015973.5(GAL):c.205G>A (p.Glu69Lys)GALUncertain significancecriteria provided, multiple submitters, no conflicts
475912NM_015973.5(GAL):c.263G>A (p.Arg88His)GALUncertain significancecriteria provided, multiple submitters, no conflicts
475916NM_015973.5(GAL):c.55T>C (p.Ser19Pro)GALUncertain significancecriteria provided, single submitter
4846799NM_015973.5(GAL):c.104C>G (p.Thr35Ser)GALUncertain significancecriteria provided, single submitter
542524NM_015973.5(GAL):c.199C>T (p.Arg67Trp)GALUncertain significancecriteria provided, multiple submitters, no conflicts
542525NM_015973.5(GAL):c.140C>T (p.Ala47Val)GALUncertain significancecriteria provided, single submitter
571157NM_015973.5(GAL):c.178C>T (p.Leu60Phe)GALUncertain significancecriteria provided, multiple submitters, no conflicts
574523NM_015973.5(GAL):c.43G>A (p.Ala15Thr)GALUncertain significancecriteria provided, multiple submitters, no conflicts
578366NM_015973.5(GAL):c.137-2A>GGALUncertain significancecriteria provided, single submitter
642760NC_000011.10:g.68685594delGALUncertain significancecriteria provided, single submitter
644385NM_015973.5(GAL):c.166G>A (p.Asp56Asn)GALUncertain significancecriteria provided, single submitter
831647NC_000011.10:g.(?68684904)(68691007_?)dupGALUncertain significancecriteria provided, single submitter
837648NM_015973.5(GAL):c.335C>G (p.Pro112Arg)GALUncertain significancecriteria provided, single submitter
838372NM_015973.5(GAL):c.28G>A (p.Ala10Thr)GALUncertain significancecriteria provided, multiple submitters, no conflicts
850500NM_015973.5(GAL):c.16G>A (p.Ala6Thr)GALUncertain significancecriteria provided, multiple submitters, no conflicts
851157NM_015973.5(GAL):c.223+3G>AGALUncertain significancecriteria provided, single submitter
851392NM_015973.5(GAL):c.311C>T (p.Ala104Val)GALUncertain significancecriteria provided, single submitter
863573NM_015973.5(GAL):c.191G>A (p.Arg64Gln)GALUncertain significancecriteria provided, single submitter
864078NM_015973.5(GAL):c.316G>A (p.Asp106Asn)GALUncertain significancecriteria provided, multiple submitters, no conflicts
931506NM_015973.5(GAL):c.81+12G>AGALUncertain significancecriteria provided, single submitter

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 1 · Orphanet: 0 · 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
GALLimitedAutosomal dominantfamilial temporal lobe epilepsy 8

Cohort genes → proteins

1 cohort genes, 1 distinct canonical proteins.

Evidence partition

SubsetGenes
multi_evidence1

Cohort genes (full)

SymbolHGNCEnsemblUniProtNameEvidence
GALHGNC:4114ENSG00000069482P22466Galanin peptidesgencc,clinvar

Cohort function summary

Lead sentence per gene, UniProt-curated.

SymbolProtein nameFunction (lead sentence)
GALGalanin peptidesEndocrine hormone of the central and peripheral nervous systems that binds and activates the G protein-coupled receptors GALR1, GALR2, and GALR3.

Protein-family classification

Druggable: 0 · Difficult: 0 · Unknown: 1 · Druggable fraction: 0.0

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
Other/Unknown11.8×0.558

Per-gene assignment

SymbolFamilyDruggable?ECInterPro (top 3)
GALOther/UnknownnoGalanin, Galanin_pre, GMAP

Expression context

Cohort genes with no expression data: 0.

1 cohort gene 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)1
unknown0

Top tissues across cohort

TissueCohort genes
adenohypophysis1
pituitary gland1
upper leg skin1

Per-gene tissue summary (top 30)

SymbolBgee breadthFANTOM5 breadthSCXATop tissues
GAL164ubiquitousmarkeradenohypophysis, pituitary gland, upper leg skin

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

SymbolInteractor count
GAL1,160

Structural data

PDB: 1 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

SymbolUniProtPDB entries
GALP2246610

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 2. Enrichment computed across 1 evidence-associated genes (1 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 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

PathwayCohort genesFoldFDRSample cohort genes
Peptide ligand-binding receptors174.2×0.026GAL
G alpha (i) signalling events139.0×0.026GAL

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO termCohort genesFoldFDRSample cohort genes
regulation of glucocorticoid metabolic process18426.0×0.001GAL
parental behavior18426.0×0.001GAL
positive regulation of timing of catagen15617.3×0.001GAL
positive regulation of cortisol secretion14213.0×0.001GAL
negative regulation of lymphocyte proliferation13370.4×0.001GAL
obsolete protein kinase A signaling11404.3×0.002GAL
behavioral response to ethanol11203.7×0.002GAL
response to peptide11123.5×0.002GAL
drinking behavior1991.3×0.002GAL
response to immobilization stress1732.7×0.003GAL
insulin secretion1432.1×0.004GAL
response to estrogen1343.9×0.005GAL
response to insulin1230.8×0.007GAL
locomotory behavior1179.3×0.008GAL
neuropeptide signaling pathway1172.0×0.008GAL
response to xenobiotic stimulus169.1×0.018GAL
positive regulation of apoptotic process156.7×0.021GAL
nervous system development145.9×0.024GAL
signal transduction116.1×0.066GAL
positive regulation of transcription by RNA polymerase II114.9×0.067GAL

Therapeutics

Drug target analysis

Approved (phase 4): 0 · Phase ≥3: 0 · Phased (≥1): 0 · Undrugged: 1

Druggability breadth: 0 of 1 evidence-associated genes (0%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Top cohort targets by molecule count

SymbolMoleculesMax phase
GAL00

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 0.

Pharmacogenomics

Cohort genes with a PharmGKB record: 1; with CPIC/DPWG dosing guidelines: 0.

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

Chemical tractability of cohort targets

0 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.

Druggability pyramid

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

TierDefinitionGenesSymbols
AApproved (phase 4 drug)0
BPhased (≥1) drug, not yet approved0
CDruggable family + PDB, no drug0
DDruggable family + AlphaFold only, no drug0
EDifficult family or no structure, no drug1GAL

Undrugged target profiles

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

SymbolChEMBL assaysDrugged partners (top 3)
GAL0

Clinical trials & evidence

Clinical trials

Clinical trials: 0.

  • Cohort genes: GAL