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Recognized gene
LOXHD1
Normalized c.HGVS
c.1164C>G, c.1208C>T, c.1419C>G, c.1476G>T, c.1639C>T and 45 more
Normalized p.HGVS
p.(Ala1538Thr), p.(Ala403Val), p.(Ala679Thr), p.(Ala763Val), p.(Arg1000Trp) and 44 more
Matching records
208
PM3-positive records
20

Results are ordered by PM3-relevant evidence first. Reviewed source only indicates whether main text, supplementary material, or both were reviewed; it is not the evidence conclusion.

Download current query results as TSV
Gene Variant Evidence PM3 context Publication Reviewed source Actions
LOXHD1 NM_001384474.1:c.1828G>A Phase-confirmed PM3 evidence
High confidence
Confirmed in trans with c.2825-2827delAGA
context: Confirmed in trans
31709873
A novel LOXHD1 variant in a Chinese couple with hearing loss.
The Journal of international medical research, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.3874C>T Phase-unconfirmed biallelic evidence
High confidence
Homozygous for query variant
context: Homozygous evidence
40079965
Genetic and Demographic Determinants of Fuchs Endothelial Corneal Dystrophy Risk and Severity.
JAMA ophthalmology, 2025
Supplementary material
Open
LOXHD1 NM_001384474.1:c.1759C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.2438T>A; Leu813Ter; Nonsense
context: Compound heterozygous candidate
39767564
Next-Generation Sequencing of Chinese Children with Congenital Hearing Loss Reveals Rare and Novel Variants in Known and Candidate Genes.
Biomedicines, 2024
Supplementary material
Open
LOXHD1 NM_001384474.1:c.2575C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.1228C>T; p.(Gln410Ter); p.Gln410Ter
context: Compound heterozygous candidate
35682719
Searching for the Molecular Basis of Partial Deafness.
International journal of molecular sciences, 2022
Main article
Open
LOXHD1 NM_001384474.1:c.1751C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.5815G > A; p.D1939 N
context: Compound heterozygous candidate
35875410
Mutations in LOXHD1 gene can cause auditory neuropathy spectrum disorder.
Otolaryngology case reports, 2021
Main article
Open
LOXHD1 NM_001384474.1:c.1191G>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.5813G>A; p.(Arg1938His); p.Arg1938His
context: Compound heterozygous candidate
33484326
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Journal of neurology, 2021
Main article
Open
LOXHD1 NM_001384474.1:c.4690C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.2054G > A; c.2054G>A; p.Arg685His
context: Compound heterozygous candidate
33484326
Contribution of nuclear and mitochondrial gene mutations in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.
Journal of neurology, 2021
Main article
Open
LOXHD1 NM_001384474.1:c.6322G>A Phase-unconfirmed biallelic evidence
High confidence
Homozygous for query variant
context: Homozygous evidence
32682410
Whole exome sequencing identified mutations causing hearing loss in five consanguineous Pakistani families.
BMC medical genetics, 2020
Main article
Open
LOXHD1 NM_001384474.1:c.277G>A Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.1255+3A>G
context: Compound heterozygous candidate
32149082
Five Novel Mutations in LOXHD1 Gene Were Identified to Cause Autosomal Recessive Nonsyndromic Hearing Loss in Four Chinese Families.
BioMed research international, 2020
Main article
Open
LOXHD1 NM_001384474.1:c.1751C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.[5815G > A]; p.[D1939N]
context: Compound heterozygous candidate
31547530
Mutational Spectrum and Clinical Features of Patients with LOXHD1 Variants Identified in an 8074 Hearing Loss Patient Cohort.
Genes, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.1828G>A Phase-unconfirmed biallelic evidence
High confidence
Homozygous for query variant
context: Homozygous evidence
31547530
Mutational Spectrum and Clinical Features of Patients with LOXHD1 Variants Identified in an 8074 Hearing Loss Patient Cohort.
Genes, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.1843C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.[3281A > G]; p.[D1094G]
context: Compound heterozygous candidate
31547530
Mutational Spectrum and Clinical Features of Patients with LOXHD1 Variants Identified in an 8074 Hearing Loss Patient Cohort.
Genes, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.5794C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.1270 + 4A > C
context: Compound heterozygous candidate
31547530
Mutational Spectrum and Clinical Features of Patients with LOXHD1 Variants Identified in an 8074 Hearing Loss Patient Cohort.
Genes, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.6055G>A Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.1730T > G; c.5944C > T; L577R; +1 more
context: Compound heterozygous candidate
31547530
Mutational Spectrum and Clinical Features of Patients with LOXHD1 Variants Identified in an 8074 Hearing Loss Patient Cohort.
Genes, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.1751C>T Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.5815G > A
context: Compound heterozygous candidate
30760222
Whole-exome sequencing identifies a novel missense variant within LOXHD1 causing rare hearing loss in a Chinese family.
BMC medical genetics, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.2696G>A Phase-unconfirmed biallelic evidence
Needs review
Possible compound heterozygous with c.3834G > C; c.5934C > T
context: Compound heterozygous candidate
30760222
Whole-exome sequencing identifies a novel missense variant within LOXHD1 causing rare hearing loss in a Chinese family.
BMC medical genetics, 2019
Main article
Open
LOXHD1 NM_001384474.1:c.4130G>A Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with p.Ala138Glu
context: Compound heterozygous candidate
30242206
In Vivo Electrocochleography in Hybrid Cochlear Implant Users Implicates TMPRSS3 in Spiral Ganglion Function.
Scientific reports, 2018
Main article
Open
LOXHD1 NM_001384474.1:c.1751C>T Phase-unconfirmed biallelic evidence
High confidence
Confirmed in trans with c.5815G>A; p.D1939N
context: Confirmed in trans
30123251
Genetic Etiology Study of Ten Chinese Families with Nonsyndromic Hearing Loss.
Neural plasticity, 2018
Main article
Open
LOXHD1 NM_001384474.1:c.3979T>A Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.3371G>A; p.Arg1124His
context: Compound heterozygous candidate
27246798
Targeted genomic enrichment and massively parallel sequencing identifies novel nonsyndromic hearing impairment pathogenic variants in Cameroonian families.
Clinical genetics, 2016
Main article
Open
LOXHD1 NM_001384474.1:c.6659G>A Phase-unconfirmed biallelic evidence
High confidence
Possible compound heterozygous with c.6107C>T; p.A2036V
context: Compound heterozygous candidate
26496393
Dependable and Efficient Clinical Molecular Diagnosis of Chinese RP Patient with Targeted Exon Sequencing.
PloS one, 2015
Supplementary material
Open