Immunochemical and Serological Properties of
Biphasic Haemolysins

Hart, Mia van der; Giessen, Mark van der; Veer, Marga van der; Peetoom, F.; Loghem, J.J. van

doi:10.1159/000464944

Cited by 3 publications

(3 citation statements)

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“…These findings have been confirmed by van Loghem et al [13], but it appears that not all antibodies demonstrate this specificity [14]. The antibody is usually a 7S gamma globulin, although some heterogeneity has been noted [6,13].…”

mentioning

confidence: 70%

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Membrane Destruction in Paroxysmal Cold Hemoglobinuria

Scott¹,

Weed²,

Swisher³

1967

Vox Sanguinis

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mentioning

confidence: 70%

“…However, LEVINE et al [7] have shown that the antibody has distinct anti-P -t P,(Tja) specificity. These findings have been confirmed by VAN LOGHEM et al [13], but it appears that not all antibodies demonstrate this specificity [14]. The antibody is usually a 7s gamma globulin, although some heterogeneity has been noted [6, 131.…”

mentioning

confidence: 80%

Membrane Destruction in Paroxysmal Cold Hemoglobinuria

Scott¹,

Weed²,

Swisher³

1967

Vox Sanguinis

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“…ABCG2 mediates PPIX export from red blood cells, the major site of haem synthesis 11 12 . Plasma membrane ABCB6 defines the Langereis (Lan) antigen 15 , which can cause severe haemolytic transfusion reactions and haemolytic disease of the fetus and newborn 19 . Lan(−) individuals are typically asymptomatic, thus the physiological function of plasma membrane ABCB6 is unknown.…”

Section: Resultsmentioning

confidence: 99%

The severity of hereditary porphyria is modulated by the porphyrin exporter and Lan antigen ABCB6

et al. 2016

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Hereditary porphyrias are caused by mutations in genes that encode haem biosynthetic enzymes with resultant buildup of cytotoxic metabolic porphyrin intermediates. A long-standing open question is why the same causal porphyria mutations exhibit widely variable penetrance and expressivity in different individuals. Here we show that severely affected porphyria patients harbour variant alleles in the ABCB6 gene, also known as Lan, which encodes an ATP-binding cassette (ABC) transporter. Plasma membrane ABCB6 exports a variety of disease-related porphyrins. Functional studies show that most of these ABCB6 variants are expressed poorly and/or have impaired function. Accordingly, homozygous disruption of the Abcb6 gene in mice exacerbates porphyria phenotypes in the Fechm1Pas mouse model, as evidenced by increased porphyrin accumulation, and marked liver injury. Collectively, these studies support ABCB6 role as a genetic modifier of porphyria and suggest that porphyrin-inducing drugs may produce excessive toxicities in individuals with the rare Lan(−) blood type.

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