Effects of Sulfhydryl Inhibition on Red Blood Cells. Ii. Studies in Vivo*

Jacob, Harry S.; Jandl, James H.

doi:10.1172/jci104607

Cited by 159 publications

(54 citation statements)

References 24 publications

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“…The red cells acquire the following characteristics: spheroidal shape, increased osmotic fragility, decreased deformability, decreased lifespan, and splenic sequestration (65)(66)(67). In previous studies, we found that mild chemical oxidation of spectrin markedly impaired its ability to bind protein 4.1 (41), similar to the defect observed for the HS(Sp-4.…”

Section: Discussionmentioning

confidence: 99%

Abnormal oxidant sensitivity and beta-chain structure of spectrin in hereditary spherocytosis associated with defective spectrin-protein 4.1 binding.

Becker¹,

Morrow²,

Lux³

1987

J. Clin. Invest.

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Hereditary spherocytosis (HS) is an inherited disorder of erythrocyte shape associated with spectrin deficiency and hemolytic anemia. In a subset of patients with the autosomal dominant form of HS, spectrin displays a reduced capacity to bind protein 4.1 and, therefore, actin; both functions that are critical to the membrane skeleton. A specific structural defect has not been identified in the spectrin from these patients. Chymotryptic digestion of the isolated spectrin chains shows impaired cleavage of the distal peptide of the beta subunit, the beta IV domain. In previous work, we have shown that mild oxidation markedly diminishes the binding capacity of normal spectrin for protein 4

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Section: Discussionmentioning

confidence: 99%

Abnormal oxidant sensitivity and beta-chain structure of spectrin in hereditary spherocytosis associated with defective spectrin-protein 4.1 binding.

Becker¹,

Morrow²,

Lux³

1987

J. Clin. Invest.

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“…In other instances, the spleen, despite its modest blood flow, is the dominant sequestering organ. Thus a variety of pathological red cells (8)(9)(10)(11) and red cells mildly altered by chemical (3)(4)(5)12), physical (5), or immune (1, 2) agents are susceptible to selective splenic sequestration and destruction. Comparatively little is known of the participation of the lungs and bone marrow in particle clearance.…”

mentioning

confidence: 99%

“…In a report (28) (31). In both cases, concentrations of antibody or inhibitor were chosen that gave pronounced splenic but little hepatic uptake of the altered cells (12,13). Three hours after injection, at a time when approximately 50% of the labeled cells had been sequestered, the animals were sacrificed.…”

mentioning

confidence: 99%

Regulation of Spleen Growth and Sequestering Function*

Jacob¹,

MacDonald²,

Jandl³

1963

J. Clin. Invest.

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“…The enzymes of GSH synthesis were measured in hemolysates from 25 FIGURE 2 Effect of cell age on the enzymes of GSH synthesis. Blood samples from patients with acquired hemolytic anemia and from normal subjects were fractionated into "reticulocyte-rich" and "reticulocyte-poor" fractions by differential centrifugation.…”

Section: Resultsmentioning

confidence: 99%

“…In this situation de novo GSH synthesis may be important in restoring cellular levels of GSH. Jacob and Jandl (25) demonstrated that, after 90% of intracellular GSH was alkylated in vitro with N-ethylmaleimide, erythrocytes survived normally when transfused back to the donors. This may be explained by the fact that GSH synthesis could restore normal levels of GSH in these cells within 1 hr, assuming that one can extrapolate in vitro enzyme activity to the in vivo situation.…”

Section: Discussionmentioning

confidence: 99%