Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
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Drug
Enzyme
Compound
Query: UMLS:C0039730 (
thalassemia
)
10,305
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Four hydrolases, beta-galactosidase, beta-glucuronidase, beta-N-acetylglucosaminidase and acid phosphatase were examined in red blood cells (RBC) of normal donors and patients with homozygous beta-
thalassaemia
. Highly sensitive fluorimetric substrates were used to determine the specific activities of these enzymes. In order to avoid contamination by lysosomal activities derived from white blood cells (WBC), the mature
RBV
were separated from other blood elements by cellulose chromatography. The hydrolase activities in normal RBC were detected only in their plasma membranes and were found to be considerably lower than in WBC or platelets. In thalassaemic RBC, hydrolase activities were present in both plasma membranes and in the soluble fraction. The normoblast fraction contributed most of the hydrolase activity found in these preparations, suggesting the presence of lysosomal particles in thalassaemic RBC. No differences in the enzymatic activities were found when purified membranes of mature RBC from thalassemic and normal preparations were compared. The origin and roles of these hydrolytic enzymes in normal and thalassaemic RBC membranes are not known.
...
PMID:"Lysosomal" enzyme activities in red blood cells of normal individuals and patients with homozygous beta-thalassaemia. 11 37
Raman microspectroscopy was applied to monitor porphyrin perturbation associated with the tense-relaxed (T --> R) state transition of hemoglobin (Hb) within a single red blood cell. The spectra exhibited dramatic changes in the spin state region (1650-1500 cm(-1)) and the methine C-H deformation region (1250-1200 cm(-1)) between the 27- and 54-min interval following reoxygenation. Principal components analysis revealed a sigmoidal curve when the PC1 scores versus time were plotted. The inflection point on the curve corresponded to the T --> R transition point spectrum, indicating the cell is composed of Hb in a mixture of spin states. The results of this work illustrate the dynamics of porphyrin perturbation during erythrocyte respiration and may have applications in the diagnosis and treatment of red blood cell disorders including
thalassemia
and sickle cell anemia.
Biopolymers
2002
PMID:Micro-Raman characterization of high- and low-spin heme moieties within single living erythrocytes. 1201 42
Matriptase-2 plays a pivotal role in keeping iron concentrations within a narrow physiological range in humans. The opportunity to reduce matriptase-2 proteolytic activity may open a novel possibility to treat iron overload diseases, such as hereditary hemochromatosis and
thalassemia
. Here, we present 23 new analogues of trypsin inhibitor SFTI-1 designed to inhibit human matriptase-2. Influence of the modifications Gly1Lys, Ile10Arg, and Phe12His, as well as the introduction of Narg in P1 or P1 and P4 positions were examined. Selected peptides were further analyzed, together with previously reported peptides, for their inhibitory activity against related human proteases, that are, matriptase-1, plasmin, thrombin and trypsin. A highly potent inhibitor of matriptase-2, the bicycylic [Arg
5
, Arg
10
, His
12
]SFTI-1, with a K
i
value of 15 nm was obtained.
Biopolymers
2017 Nov
PMID:Design and chemical syntheses of potent matriptase-2 inhibitors based on trypsin inhibitor SFTI-1 isolated from sunflower seeds. 2855 56
