Hemoglobin as a Direct Inhibitor of Cartilage Growth In Vitro

Vassilopoulou‐Sellin, Rena; Oyedeji, Caroline O.; Foster, Paulette; Thompson, M.Malynn; Saman, Naguib

doi:10.1055/s-2007-1009138

Cited by 19 publications

(5 citation statements)

References 9 publications

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“…Indeed, improved height attainment has been described in hypertransfused thalassaemic patients (Kattamis et al, 1970;Giardina et al, 1990). Moreover, based on experimental observations, it has recently been suggested that increased amounts of circulating free haemoglobin may inhibit cartilage growth, thus accounting for the growth retardation associated with chronic haemolytic conditions (Vassilopoulou-Sellin et al, 1989). Finally, evidence has been provided that early administration of desferrioxamine, i.e.…”

Section: Discussionmentioning

confidence: 99%

Treatment with biosynthetic growth hormone of short thalassaemic patients with impaired growth hormone secretion

Scacchi

Danes

Martin

et al. 1991

Clinical Endocrinology

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Section: Discussionmentioning

confidence: 99%

Treatment with biosynthetic growth hormone of short thalassaemic patients with impaired growth hormone secretion

Scacchi

Danes

Martin

et al. 1991

Clinical Endocrinology

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“…It has been observed that hemoglobin, heme, and bilirubin have an inhibitory effect on cartilage metabolism and growth in vitro although the molecular mechanism is not clear [44–46]. FLVCR1 is expressed at high levels during development in the yolk sac and placenta [5].…”

Section: Diamond Blackfan Anemia As a Putative Disorder Of Heme Mementioning

confidence: 99%

Diamond Blackfan Anemia at the Crossroad between Ribosome Biogenesis and Heme Metabolism

Chiabrando

Tolosano

2010

Advances in Hematology

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Diamond-Blackfan anemia (DBA) is a rare, pure red-cell aplasia that presents during infancy. Approximately 40% of cases are associated with other congenital defects, particularly malformations of the upper limb or craniofacial region. Mutations in the gene coding for the ribosomal protein RPS19 have been identified in 25% of patients with DBA, with resulting impairment of 18S rRNA processing and 40S ribosomal subunit formation. Moreover, mutations in other ribosomal protein coding genes account for about 25% of other DBA cases. Recently, the analysis of mice from which the gene coding for the heme exporter Feline Leukemia Virus subgroup C Receptor (FLVCR1) is deleted suggested that this gene may be involved in the pathogenesis of DBA. FLVCR1-null mice show a phenotype resembling that of DBA patients, including erythroid failure and malformations. Interestingly, some DBA patients have disease linkage to chromosome 1q31, where FLVCR1 is mapped. Moreover, it has been reported that cells from DBA patients express alternatively spliced isoforms of FLVCR1 which encode non-functional proteins. Herein, we review the known roles of RPS19 and FLVCR1 in ribosome function and heme metabolism respectively, and discuss how the deficiency of a ribosomal protein or of a heme exporter may result in the same phenotype.

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“…129,130 Hyposecretion of adrenal animproved, compared with the prognosis reported 30 years drogen, 106,107 delay in pubertal development itself, 131,132 zinc ago in similar patients. 68 A recent study reported that irondeficiency, [133][134][135] and free-hemoglobin-induced inhibition of induced cardiac disease was fatal in most patients in whom cartilage growth 136 have also been implicated in impairment body iron burdens remained high, but that extended survivals of growth in patients with thalassemia major.…”

Section: Clinica L Complications Of Iron Overload Andmentioning

confidence: 99%

Iron-Chelating Therapy and the Treatment of Thalassemia

Olivieri

Brittenham

1997

Blood

977

549

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improved control of body iron burden, 6,7 compared with a transfusion schedule (termed ''supertransfusion'') in which HE LAST 3 decades have witnessed profound changes in the management of patients with thalassemia major. Regular red blood cell (RBC) transfusions eliminate the baseline hemoglobins are permitted to exceed 11 g/dL. 8 complications of anemia and compensatory bone marrow Type of RB C Concentrates (BM) expansion, permit normal development throughout childhood, and extend survival. 1 In parallel, transfusions re-Early studies of the use of neocytes, or young RBCs, sult in a ''second disease'' while treating the first, 2 that predicted that prolonged survival in vivo of these concenof the inexorable accumulation of tissue iron that, without trates should reduce the RBC mass required to maintain treatment, is fatal in the second decade of life. Further alterappropriate baseline hemoglobin concentrations. 8-11 Clinical ing the prognosis of thalassemia major over the last 20 years investigations confirmed that an extension of transfusion inhas been progress in the development of iron-chelating therterval of 13% to 25% in thalassemia could be achieved with apy for iron overload. Deferoxamine mesylate, first introthe use of neocytes. 12-16 A recent study found that a 15% duced in short-term studies in iron-loaded patients in the extension of transfusion interval during administration of early 1960s, gained acceptance as standard therapy over a neocyte concentrates, expected to minimally reduce the redecade later in countries able to support the high costs of quirement for iron chelation therapy, could be achieved but this therapy. Twenty years later, extended survival free of at the cost of an increased exposure to donated units and a iron-induced complications, and dramatically improved fivefold increment in preparation expenses over those of quality of life, are observed in well-chelated patients. Indeed, standard concentrates. 16 Hence, the use of neocytes should over this period, iron-chelating therapy for thalassemia major have a limited impact on the long-term management of most has resulted in one of the most dramatic alterations in morchronically transfused patients. In contrast, the use of autobidity and mortality associated with a genetic disease. In this mated RBC exchange transfusions in regularly transfused review the experience gained in the use of deferoxamine, patients with sickle cell disease has been reported to substanthe benefits of and problems associated with this agent in tially reduce transfusional iron accumulation, permitting rethe treatment and prevention of iron overload, and recent duction in the intensity of chelation therapy. 17 Pilot studies progress in the development of orally effective iron-chelating in thalassemia major 18 suggest that a similar approach may drugs will be reviewed. warrant careful evaluation in this disorder.

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Hemoglobin as a Direct Inhibitor of Cartilage Growth In Vitro

Cited by 19 publications

References 9 publications

Treatment with biosynthetic growth hormone of short thalassaemic patients with impaired growth hormone secretion

Treatment with biosynthetic growth hormone of short thalassaemic patients with impaired growth hormone secretion

Diamond Blackfan Anemia at the Crossroad between Ribosome Biogenesis and Heme Metabolism

Iron-Chelating Therapy and the Treatment of Thalassemia

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