A Rare Alpha-Globin Mutation: Detailed Analysis of the Hb Rogliano Variant (α1 Cod 108 Thr→Asn)

α-Thalassemia is an autosomal recessive disorder characterized by reduced synthesis of α-globin chains, most commonly due to deletions within the α-globin gene cluster. Non-deletional variants are less common and contribute to the molecular heterogeneity of the disease. Advances in long-read sequencing have improved the analysis of complex genomic regions, including highly homologous genes such as HBA1 and HBA2. Targeted long-range PCR amplification of the HBA1 and HBA2 genes was followed by long-read sequencing on the PromethION 24 platform (Oxford Nanopore Technologies). Subsequently, the sequencing data were analyzed and visualized using the Integrative Genomics Viewer (IGV). Long-read sequencing achieved an average depth of >390x across all target regions, enabling reliable variant detection. This analysis identified five variants in the HBA1 and HBA2 genes, namely HBA1:c.326C > A (p.Thr108Asn; Hb Rogliano), HBA1:c.-41C > G, HBA2:c0.300 + 55T > G, HBA2:c0.301-24delinsCTCGGCCC, and HBA2:c.-41C > G. Based on ClinVar classification, HBA1:c.326C > A (p.Thr108Asn) was categorized as likely pathogenic, while the remaining variants were classified as benign or likely benign. To our knowledge, this is the first report of Hb Rogliano in Indonesia and highlights the utility of long-read sequencing for the molecular characterization of rare α-globin variants.

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Journal
Hemoglobin
Published
2026-08-13
DOI
https://doi.org/10.1080/03630269.2026.2715749
Primary Topic
Hemoglobinopathies and Related Disorders
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article
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article

A Rare Alpha-Globin Mutation: Detailed Analysis of the Hb Rogliano Variant (α1 Cod 108 Thr→Asn)

Niken Satuti Nur Handayani, Tri Ratnaningsih, Indra Lesmana, Alifia Nur Hidayah et al.
Hemoglobin
Hemoglobinopathies and Related Disorders
article

A Rare Alpha-Globin Mutation: Detailed Analysis of the Hb Rogliano Variant (α1 Cod 108 Thr→Asn)

Niken Satuti Nur Handayani, Tri Ratnaningsih, Indra Lesmana, Alifia Nur Hidayah, Nur Imma Fatimah Harahap, Nafis Muhimmatul ‘Ulya, Vincentius SW Budhyanto
article en

Abstract

α-Thalassemia is an autosomal recessive disorder characterized by reduced synthesis of α-globin chains, most commonly due to deletions within the α-globin gene cluster. Non-deletional variants are less common and contribute to the molecular heterogeneity of the disease. Advances in long-read sequencing have improved the analysis of complex genomic regions, including highly homologous genes such as HBA1 and HBA2. Targeted long-range PCR amplification of the HBA1 and HBA2 genes was followed by long-read sequencing on the PromethION 24 platform (Oxford Nanopore Technologies). Subsequently, the sequencing data were analyzed and visualized using the Integrative Genomics Viewer (IGV). Long-read sequencing achieved an average depth of >390x across all target regions, enabling reliable variant detection. This analysis identified five variants in the HBA1 and HBA2 genes, namely HBA1:c.326C > A (p.Thr108Asn; Hb Rogliano), HBA1:c.-41C > G, HBA2:c0.300 + 55T > G, HBA2:c0.301-24delinsCTCGGCCC, and HBA2:c.-41C > G. Based on ClinVar classification, HBA1:c.326C > A (p.Thr108Asn) was categorized as likely pathogenic, while the remaining variants were classified as benign or likely benign. To our knowledge, this is the first report of Hb Rogliano in Indonesia and highlights the utility of long-read sequencing for the molecular characterization of rare α-globin variants.

Hemoglobin
Universitas Gadjah Mada (ID), Sanata Dharma University (ID)
Openalex Percentile: Top 10%
Hemoglobinopathies and Related Disorders
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