Deficient mitophagy pathways in sickle cell disease

نویسندگان

چکیده

Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients found 40% with elevated mitochondria levels (SS-mito+ ) in their mature red blood cells, while 60% exhibit similar compared to the AA group (SS-mito- ). The SS-mito+ are higher reticulocytosis total bilirubin levels, lower foetal haemoglobin, non-functional mitochondria. Interestingly, demonstrated decreased of mitophagy inducers, PINK1 NIX, HSP90 chaperone cells. Our results highlighted for first time an abnormal retention linked mitophagy-related proteins.

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Intracardiac Thrombosis in Sickle Cell Disease

In patients with sickle cell disease, thrombotic microangiopathy is a rare complication. Also in sickle cell disease, intracardiac thrombus formation without structural heart diseases or atrial arrhythmias is a rare phenomenon. We herein describe a 22-year-old woman, who was a known case of sickle cell-βthalassemia, had a history of recent missed abortion, and was admitted with a vaso-occlusive...

متن کامل

Neonatal Screening for Sickle Cell Disease in Southwest Iran

the risk of sickle cell complications that is a common hemoglobin disorder in Southwest Iran. This study aimed at determining the incidence of Sickle Cell Disease (SCD) and other Hemoglobinopathies in newborn being at risk based on ethnic origin. Materials and Methods: In this descriptive epidemiologic  study, between September 2013 and September 2015, 8363 newborn blood samples were test...

متن کامل

Pulmonary Spirometry Parameters in Patients with Sickle Thalassemia and Sickle Cell Disease at Shafa Hospital in Khuzestan Province-Iran

Abstract Background Prevalence of hereditary blood diseases such as sickle cell anemia, sickle thalassemia and thalassemia major are high in Khuzestan province. Sickle cell anemia and beta-thalassemia are predominantly common in Iranian Arabs. Pulmonary complications account for a large proportion of morbidity and mortality in patients with and sickle cell disease. Periodic lung function asse...

متن کامل

Sickle Cell Disease and Sickle Cell Anaemia

Sickle cell haemoglobin (HbS) results from an autosomal recessively inherited mutation in which the 17th nucleotide of the beta globin gene is changed from thymine to adenine and the amino acid glutamic acid is replaced by valine at position 6 in the beta globin chain. [1, 2]Sickle cells have a reduced deformability and are easily destroyed, causing occlusion of the microcirculation and a chron...

متن کامل

Pattern of chronic lung lesions in adults with sickle cell disease in Lagos, Nigeria

Background: The vascular response to recurrent tissue hypoxia and reperfusion following red blood cell sickling causes acute chest syndrome and chronic lung disease. The purpose of this study was to determine the pattern of chronic lung lesions and possible risk factors in sickle cell patients in lagos, Nigeria.Methods: From July 2012 to April 2013, Pulmonary function test (PFT) and chest-x-ray...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

ژورنال

عنوان ژورنال: British Journal of Haematology

سال: 2021

ISSN: ['0007-1048', '1365-2141']

DOI: https://doi.org/10.1111/bjh.17416