The Effect of Zinc Therapy on Lysosomal Inclusion Bodies in Intestinal Epithelial Cells in Acrodermatitis Enteropathica

Jones, J. G.; Elmes, Margaret E.; Aggett, Peter; Harries, J T

doi:10.1203/00006450-198305000-00009

Cited by 12 publications

(3 citation statements)

References 13 publications

(5 reference statements)

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“…The dermis may show a sparse lymphohistiocytic inflammatory infiltrate. Ultrastructurally, Paneth cells of the intestinal mucosa show inclusions, as well as pleomorphic granules, sometimes becoming confluent, giving rise to giant granules [ 216–218 ]. Some enterocytes contain large lysosomal inclusion bodies [ 216 ].…”

Section: Zinc Homeostasismentioning

confidence: 99%

Disorders of metal metabolism

Ferreira

Gahl

2017

TRD

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Trace elements are chemical elements needed in minute amounts for normal physiology. Some of the physiologically relevant trace elements include iodine, copper, iron, manganese, zinc, selenium, cobalt and molybdenum. Of these, some are metals, and in particular, transition metals. The different electron shells of an atom carry different energy levels, with those closest to the nucleus being lowest in energy. The number of electrons in the outermost shell determines the reactivity of such an atom. The electron shells are divided in sub-shells, and in particular the third shell has s, p and d sub-shells. Transition metals are strictly defined as elements whose atom has an incomplete d sub-shell. This incomplete d sub-shell makes them prone to chemical reactions, particularly redox reactions. Transition metals of biologic importance include copper, iron, manganese, cobalt and molybdenum. Zinc is not a transition metal, since it has a complete d sub-shell. Selenium, on the other hand, is strictly speaking a nonmetal, although given its chemical properties between those of metals and nonmetals, it is sometimes considered a metalloid. In this review, we summarize the current knowledge on the inborn errors of metal and metalloid metabolism.

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Section: Zinc Homeostasismentioning

confidence: 99%

Disorders of metal metabolism

Ferreira

Gahl

2017

TRD

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“…Zinc is known to be essential for normal secretion, granule formation, and the sta biliza tion and release of sec retio n in both Paneths and goblet cells (13,14). The pre sence of abnormal pancreatic ac inar cells ha s been reported in zinc-deficient rat s ( 15), Zinc is probably necessary for normal secretion in all sec retory cells (\ 3).…”

Section: Discussionmentioning

confidence: 99%

Ultrastructural Changes in the Mucosa of the Small Intestine in Patients with Geophagia (Prasad's Syndrome)

Arcasoy,

Akar,

Örs

et al. 1990

J. pediatr. gastroenterol. nutr.

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“…Histological studies on gut tissue from patients have shown that acrodermatitis enteropathica is associated with cellular abnormalities. Filamentous inclusion bodies in the cytoplasm (Mack et al, 1989;Bohane et al, 1977) and abnormal lysosomal inclusions (Jones et al, 1983) occur in the intestinal Paneth cells of patients. This pathology is considered to be a secondary effect of the disease because it disappears after zinc treatment.…”

Section: Acrodermatitis Enteropathica Acrodermatitis Enteropathica Acmentioning

confidence: 99%

Zinc deficiency and its inherited disorders -a review

Ackland

Michalczyk

2006

Genes Nutr

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Zinc is an essential trace element required by all living organisms because of its critical roles both as a structural component of proteins and as a cofactor in enzyme catalysis. The importance of zinc in human metabolism is illustrated by the effects of zinc deficiency, which include a diminished immune response, reduced healing and neurological disorders. Furthermore, nutritional zinc deficiency can be fatal in newborn or growing animals. While zinc deficiency is commonly caused by dietary factors, several inherited defects of zinc deficiency have been identified. Acrodermatitis enteropathica is the most commonly described inherited condition found in humans. In several of the few cases that have been reported, this disorder is associated with mutations in the hZIP4 gene, a member of the SLC39 family, whose members encode membranebound putative zinc transporters. Mutations in other members of this family or in different genes may account for other cases of acrodermatitis in which defects in hZIP4 have not been detected. Another inherited form of zinc deficiency occurs in the lethal milk mouse, where a mutation in ZnT4 gene, a member of the SLC30 family of transmembrane proteins results in impaired secretion of zinc into milk from the mammary gland. A similar disorder to the lethal milk mouse occurs in humans. In the few cases studied, no changes in ZnT4 orthologue, hZnT4, were detected. This, and the presence of several minor phenotypic differences between the zinc deficiency in humans and mice, suggests that the human condition is caused by defects in genes that are yet to be identified. Taking into account the fact that there are no definitive tests for zinc deficiency and that this disorder can go undiagnosed, plus the recent identification of multiple members of the SCL30 and SLC39, it is likely that mutations in other genes may underlie additional inherited disorders of zinc deficiency.

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The Effect of Zinc Therapy on Lysosomal Inclusion Bodies in Intestinal Epithelial Cells in Acrodermatitis Enteropathica

Cited by 12 publications

References 13 publications

Disorders of metal metabolism

Disorders of metal metabolism

Ultrastructural Changes in the Mucosa of the Small Intestine in Patients with Geophagia (Prasad's Syndrome)

Zinc deficiency and its inherited disorders -a review

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