Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR Variant

ABSTRACT Oculocutaneous albinism type IA (OCA1A) is a rare autosomal recessive disorder caused by variants in the TYR gene, resulting in complete loss of tyrosinase activity and absence of melanin production. In this study, we report a novel missense variant, TYR (NM_000372.5):c.143A>C (p.Gln48Pr...

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Main Authors: Raghad N. Shihab, Rusul Thabit Hamid, Mostafa Neissi, Javad Mohammadi‐Asl, Motahareh Sheikh‐Hosseini, Elaheh Nekouei
Format: Article
Language:English
Published: Wiley 2025-09-01
Series:Clinical Case Reports
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Online Access:https://doi.org/10.1002/ccr3.70818
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author Raghad N. Shihab
Rusul Thabit Hamid
Mostafa Neissi
Javad Mohammadi‐Asl
Motahareh Sheikh‐Hosseini
Elaheh Nekouei
author_facet Raghad N. Shihab
Rusul Thabit Hamid
Mostafa Neissi
Javad Mohammadi‐Asl
Motahareh Sheikh‐Hosseini
Elaheh Nekouei
author_sort Raghad N. Shihab
collection DOAJ
description ABSTRACT Oculocutaneous albinism type IA (OCA1A) is a rare autosomal recessive disorder caused by variants in the TYR gene, resulting in complete loss of tyrosinase activity and absence of melanin production. In this study, we report a novel missense variant, TYR (NM_000372.5):c.143A>C (p.Gln48Pro), found within exon 1 and mapped to chr11: g.89178096A>C (GRCh38/hg38). This variant was identified through exome sequencing in a 4‐year‐old Iranian girl from a consanguineous family presenting with features of OCA1A. This discovery expands the mutational spectrum of OCA1 and underscores the importance of genetic screening in diagnosing rare inherited disorders. Future studies involving functional assays are necessary to elucidate the molecular mechanisms of this variant and its potential impact on melanogenesis.
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issn 2050-0904
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publishDate 2025-09-01
publisher Wiley
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series Clinical Case Reports
spelling doaj-art-e5e9c642a7e34ccdb8601325858c035a2025-08-25T12:56:15ZengWileyClinical Case Reports2050-09042025-09-01139n/an/a10.1002/ccr3.70818Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR VariantRaghad N. Shihab0Rusul Thabit Hamid1Mostafa Neissi2Javad Mohammadi‐Asl3Motahareh Sheikh‐Hosseini4Elaheh Nekouei5Cancer Researches Department, Iraqi Center for Cancer and Medical Genetic Research Mustansiriyah University Baghdad IraqCancer Researches Department, Iraqi Center for Cancer and Medical Genetic Research Mustansiriyah University Baghdad IraqDepartment of Genetics, Khuzestan Science and Research Branch Islamic Azad University Ahvaz IranNoor‐Gene Genetic Laboratory Ahvaz IranNoor‐Gene Genetic Laboratory Ahvaz IranDepartment of Biomedical and Clinical Science Linköping University, University Hospital Linköping SwedenABSTRACT Oculocutaneous albinism type IA (OCA1A) is a rare autosomal recessive disorder caused by variants in the TYR gene, resulting in complete loss of tyrosinase activity and absence of melanin production. In this study, we report a novel missense variant, TYR (NM_000372.5):c.143A>C (p.Gln48Pro), found within exon 1 and mapped to chr11: g.89178096A>C (GRCh38/hg38). This variant was identified through exome sequencing in a 4‐year‐old Iranian girl from a consanguineous family presenting with features of OCA1A. This discovery expands the mutational spectrum of OCA1 and underscores the importance of genetic screening in diagnosing rare inherited disorders. Future studies involving functional assays are necessary to elucidate the molecular mechanisms of this variant and its potential impact on melanogenesis.https://doi.org/10.1002/ccr3.70818exome‐sequencingoculocutaneous albinismTYR genevariant
spellingShingle Raghad N. Shihab
Rusul Thabit Hamid
Mostafa Neissi
Javad Mohammadi‐Asl
Motahareh Sheikh‐Hosseini
Elaheh Nekouei
Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR Variant
Clinical Case Reports
exome‐sequencing
oculocutaneous albinism
TYR gene
variant
title Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR Variant
title_full Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR Variant
title_fullStr Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR Variant
title_full_unstemmed Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR Variant
title_short Genetic Diagnosis of Oculocutaneous Albinism Type 1A: A Novel TYR Variant
title_sort genetic diagnosis of oculocutaneous albinism type 1a a novel tyr variant
topic exome‐sequencing
oculocutaneous albinism
TYR gene
variant
url https://doi.org/10.1002/ccr3.70818
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