Further Evidence for a Genetic Basis of Haemoglobin H Disease from Newborn Offspring of Patients

ชื่อเรื่อง

Further Evidence for a Genetic Basis of Haemoglobin H Disease from Newborn Offspring of Patients See all items with this value

ผู้สร้าง/เจ้าของผลงาน

Na-nakorn, Supa See all items with this value
Wasi, Prawase
 See all items with this value
Pornpatkul, Malida
 See all items with this value
Pootrakul, Sa-Nga See all items with this value

หัวเรื่อง

Blood Protein See all items with this value
Electrophoresis
 See all items with this value
Hemoglobinometry
 See all items with this value
Hemoglobins, Abnormal See all items with this value
Infant, Newborn
 See all items with this value
Thalassemia genetics See all items with this value

บทคัดย่อ

HAEMOGLOBIN H disease is usually a mild to moderate, sometimes severe, thalassaemic disease, characterized by the presence of haemoglobin (Hb) H in addition to Hb A, and intraerythrocytic inclusion bodies1–3. Hb H is usually not detectable in either parent of the patients. The genetic basis of the disease was a subject for speculation until Hb H was shown to consist entirely of β-polypeptide chains4, with a molecular formula of β4, while Hb A is α2β2. It is now realized that Hb H (β4) is connected with α-thalassaemia5 which, by suppressing α-chain synthesis, leads to polymerization of the unconjugated β-chains to a tetrameric form. For the same reason Hb γ4 (Bart's) and Hb δ4 occur as a result of α-chain suppression6,7. Both haemoglobins can be detected in the people with Hb H disease.

ผู้ร่วมงาน

Department of Medicine, Faculty of Medicine and Siriraj Hospital, Mahidol University, Bangkok, Thailand See all items with this value

ปีที่ผลิตเอกสาร

1969 See all items with this value

วันที่บันทึกข้อมูล

2023-04-28

ประเภท

Article
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รูปแบบ

pdf
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ขนาดหรือจำนวนของเอกสาร

2 pages

ต้นฉบับ/แหล่งที่มาของเอกสาร

Nature ; volume 223, issue 5201, page 59-60

ภาษา

en See all items with this value

รหัส หรือตัวบ่งชี้เอกสาร

https://doi.org/10.1038/223059a0 See all items with this value

ขอบเขต

Health See all items with this value

เจ้าของลิขสิทธิ์

Springer Nature Limited See all items with this value

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Further Evidence for a Genetic Basis of Haemoglobin H Disease from Newborn Offspring of Patients.jpg

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ผลงานวิชาการ
บทความวิชาการ
Thalassemia genetics
HAEMOGLOBIN H
Newborn Offspring of Patients

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