HBA1 Chromosome 16

Hemoglobin subunit alpha 1
62 variants 62 Health Risk

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What This Gene Does
The human alpha globin gene cluster located on chromosome 16 spans about 30 kb and includes seven loci: 5'- zeta - pseudozeta - mu - pseudoalpha-1 - alpha-2 - alpha-1 - theta - 3'. The alpha-2 (HBA2) and alpha-1 (HBA1) coding sequences are identical. These genes differ slightly over the 5' untranslated regions and the introns, but they differ significantly over the 3' untranslated regions. Two alpha chains plus two beta chains constitute HbA, which in normal adult life comprises about 97% of the total hemoglobin; alpha chains combine with delta chains to constitute HbA-2, which with HbF (fetal hemoglobin) makes up the remaining 3% of adult hemoglobin. Alpha thalassemias result from deletions of each of the alpha genes as well as deletions of both HBA2 and HBA1; some nondeletion alpha thalassemias have also been reported. [provided by RefSeq, Jul 2008]
Gene Info
Gene Group
"Hemoglobin subunits|Proteins encoded by multiple genes"
Locus Type
gene with protein product
Location
16p13.3
Ensembl
ENSG00000206172
Associated Conditions (59)
HEMOGLOBIN BUFFALO
alpha Thalassemia
HEMOGLOBIN MAHIDOL
HEMOGLOBIN ASABARA
HEMOGLOBIN G (TAICHUNG)
HEMOGLOBIN Q (THAILAND)
HEMOGLOBIN KURASHIKI
HEMOGLOBIN Q
HEMOGLOBIN G (GEORGIA)
HEMOGLOBIN ST. LUKE'S
HEMOGLOBIN RUSS
HEMOGLOBIN WINNIPEG
HEMOGLOBIN O (PADOVA)
HEMOGLOBIN TWIN PEAKS
HBA1-related disorder
HEMOGLOBIN I (PHILADELPHIA)
HEMOGLOBIN I (TEXAS)
HEMOGLOBIN I
HEMOGLOBIN I (BURLINGTON)
HEMOGLOBIN I (SKAMANIA)
+39 more conditions
Key Variants
All Variants (62)
RSID Category Clinical Significance Conditions
RS35239527 Health Risk Pathogenic; other HEMOGLOBIN LOIRE, Erythrocytosis, familial
RS35672478 Health Risk Pathogenic; other HEMOGLOBIN AGHIA SOPHIA, Hemoglobin H disease, nondeletional
RS35723200 Health Risk Pathogenic; other HEMOGLOBIN ETHIOPIA, HEMOGLOBIN ROUEN, Erythrocytosis
RS63750950 Health Risk Pathogenic; other HEMOGLOBIN SASSARI, Erythrocytosis, familial
RS1181505507 Health Risk Pathogenic/Likely pathogenic alpha Thalassemia, Erythrocytosis, familial
RS1377412693 Health Risk Pathogenic/Likely pathogenic alpha Thalassemia, Hemoglobin H disease, Methemoglobinemia
RS1596573335 Health Risk Pathogenic/Likely pathogenic Erythrocytosis, familial, 7
RS1902161681 Health Risk Pathogenic/Likely pathogenic Alpha-thalassemia, Dutch type, Erythrocytosis
RS281864535 Health Risk Pathogenic/Likely pathogenic alpha Thalassemia, Heinz body anemia, Hemoglobin H disease
RS33964317 Health Risk Pathogenic/Likely pathogenic alpha Thalassemia, Heinz body anemia, Erythrocytosis
RS63750090 Health Risk Pathogenic/Likely pathogenic alpha Thalassemia, Methemoglobinemia, alpha type
RS767911847 Health Risk Pathogenic/Likely pathogenic alpha Thalassemia, Hemoglobin H disease, Methemoglobinemia
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