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Boris E Shmukler

Explore the profile of Boris E Shmukler including associated specialties, affiliations and a list of published articles. Areas
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Articles 51
Citations 937
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Recent Articles
1.
Rivera A, Nasburg J, Shim H, Shmukler B, Kitten J, Wohlgemuth J, et al.
Am J Physiol Cell Physiol . 2022 Jul; 323(3):C694-C705. PMID: 35848620
Red cell volume is a major determinant of HbS concentration in sickle cell disease. Cellular deoxy-HbS concentration determines the delay time, the interval between HbS deoxygenation and deoxy-HbS polymerization. Major...
2.
Shmukler B, Rivera A, Nishimura K, Hsu A, Wohlgemuth J, Dlott J, et al.
Physiol Rep . 2022 Mar; 10(5):e15186. PMID: 35274823
Investigation of erythrocytes from spontaneous or engineered germ-line mutant mice has been instrumental in characterizing the physiological functions of components of the red cell cytoskeleton and membrane. However, the red...
3.
Vandorpe D, Rivera A, Shmukler B, Wohlgemuth J, Dlott J, Snyder L, et al.
Blood Cells Mol Dis . 2021 Nov; 92:102619. PMID: 34768199
The molecular identity of Psickle, the deoxygenation-activated cation conductance of the human sickle erythrocyte, remains unknown. We observed in human sickle red cells that inhibitors of TRPA1 and TRPV1 inhibited...
4.
Vandorpe D, Shmukler B, Ilboudo Y, Bhasin S, Thomas B, Rivera A, et al.
Haematologica . 2021 Jun; 106(10):2759-2762. PMID: 34109777
No abstract available.
5.
Jobst-Schwan T, Klambt V, Tarsio M, Heneghan J, Majmundar A, Shril S, et al.
Kidney Int . 2020 Jan; 97(3):567-579. PMID: 31959358
Distal renal tubular acidosis is a rare renal tubular disorder characterized by hyperchloremic metabolic acidosis and impaired urinary acidification. Mutations in three genes (ATP6V0A4, ATP6V1B1 and SLC4A1) constitute a monogenic...
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Shmukler B, Rivera A, Bhargava P, Nishimura K, Kim E, Hsu A, et al.
Blood Cells Mol Dis . 2019 Dec; 81:102389. PMID: 31835175
β-thalassemia (β-Thal) is caused by defective β-globin production leading to globin chain imbalance, aggregation of free alpha chain in developing erythroblasts, reticulocytes, and mature circulating red blood cells. The hypochromic...
8.
Shmukler B, Rivera A, Bhargava P, Nishimura K, Hsu A, Kim E, et al.
Blood Cells Mol Dis . 2019 Jul; 79:102346. PMID: 31352162
Excessive red cell dehydration contributes to the pathophysiology of sickle cell disease (SCD). The densest fraction of sickle red cells (with the highest corpuscular hemoglobin concentration) undergoes the most rapid...
9.
Rivera A, Vandorpe D, Shmukler B, Andolfo I, Iolascon A, Archer N, et al.
Am J Physiol Cell Physiol . 2019 May; 317(2):C287-C302. PMID: 31091145
Hereditary xerocytosis (HX) is caused by missense mutations in either the mechanosensitive cation channel PIEZO1 or the Ca-activated K channel KCNN4. All HX-associated KCNN4 mutants studied to date have revealed...
10.
Gnanasambandam R, Rivera A, Vandorpe D, Shmukler B, Brugnara C, Snyder L, et al.
Hemasphere . 2019 Mar; 2(5):e55. PMID: 30887001
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