Biliary copper excretion by hepatocyte lysosomes in the rat. Major excretory pathway in experimental copper overload.

Gross, John B.; Myers, Brent; Kost, Louis J.; Kuntz, Susan M.; LaRusso, Nicholas F.

doi:10.1172/jci113873

Cited by 107 publications

(66 citation statements)

References 27 publications

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“…Table 3 summarizes the copper metabolism and lysosomal alterations in our study of endogenous copper overload in LEC rats as well as in previous studies on exogenous/experimental copper overload by Gross et al (1989) and Harada et al (1993). Two points should be emphasized.…”

Section: Discussionmentioning

confidence: 94%

“…Lysosomes play a pivotal role in intracellular digestion and storage of various macromolecules (LaRusso 1989; Yamazaki and LaRusso 1989) and, in he- LaRusso 1989) and the alterations of this pathway have been extensively studied in experimental copper overload (Gross et al 1989;Harada et al 1993). In contrast, the studies on lysosomal exocytosis into bile during spontaneous copper accumulation have been limited mainly due to the rarity of the disease, the difficulty in obtaining bile samples from patients, and the limited access to animal model.…”

Section: Discussionmentioning

confidence: 99%

“…Bile collection A complete biliary fistula was created between 6 a.m. and 8 a.m., because diurnal variations of biliary copper and lysosomal excretion have been reported (Gross et al 1989;Nakano et al 1990). Under pentobarbital anesthesia (50 mg/kg i.p.…”

Section: Animalsmentioning

confidence: 99%

“…Structural integrity of the hepatic lysosomes was assessed by latency measurement of NAG in E-fraction (Gross et al 1989). The latency was calculated using the following equation: latency (%) _ (total activity-free activity)/total activity x 100.…”

Section: Assessment Of Structural Integrity Of Hepatic Lysosomesmentioning

confidence: 99%

“…However, information regarding the actual alteration of the "lysosome-to-bile hepatic excretory pathway (LaRusso 1989; Yamazaki and LaRusso 1989)" in spontaneous copper accumulation is fragmentary (Goldfischer 1963;Frommer 1974). Furthermore, previous experimental studies (Gross et al 1989; Harada et al 1993), investigating the relationship between biliary copper excretion and lysosomal exocytosis, equivocally used animals exogenously overloaded with copper which may potentially differ from spontaneous copper overload.…”

mentioning

confidence: 99%

See 4 more Smart Citations

Impaired Biliary Excretion of Copper and Lysosomal Enzymes in Long-Evans Cinnamon Rat.

Abe

Yamazaki

Takikawa

et al. 1994

Tohoku J. Exp. Med.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Animalsmentioning

confidence: 99%

Section: Assessment Of Structural Integrity Of Hepatic Lysosomesmentioning

confidence: 99%

mentioning

confidence: 99%

See 3 more Smart Citations

Impaired Biliary Excretion of Copper and Lysosomal Enzymes in Long-Evans Cinnamon Rat.

Abe

Yamazaki

Takikawa

et al. 1994

Tohoku J. Exp. Med.

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Molecular mechanisms of copper metabolism and the role of the Menkes disease protein

Harrison

Dameron

1999

J. Biochem. Mol. Toxicol.

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Menkes disease is an X‐linked, recessive disorder of copper metabolism that occurs in approximately 1 in 200,000 live births. The condition is characterized by skeletal abnormalities, severe mental retardation, neurologic degeneration, and patient mortality in early childhood. The symptoms of Menkes disease result from a deficiency of serum copper and copper‐dependent enzymes. A candidate gene for the disease has been isolated and designated MNK. The MNK gene codes for a P‐type cation transporting ATPase, based on homology to known P‐type ATPases and in vitro experimentation. cDNA clones of MNK in Menkes patients show diminished or absented hybridization in northern blot experiments. The Menkes protein functions to export excess intracellular copper and activates upon Cu(I) binding to the six metal‐binding repeats in the amino‐terminal domain. The loss of Menkes protein activity blocks the export of dietary copper from the gastrointestinal tract and causes the copper deficiency associated with Menkes disease. Each of the Menkes protein amino‐terminal repeats contains a conserved ‐X‐Met‐X‐Cys‐X‐X‐Cys‐ motif (where X is any amino acid). These metal‐binding repeats are conserved in other cation exporting ATPases involved in metal metabolism and in proteins involved in cellular defense against heavy metals in both prokaryotes and eukaryotes. An overview of copper metabolism in humans and a discussion of our understanding of the molecular basis of cellular copper homeostasis is presented. This forms the basis for a discussion of Menkes disease and the protein deficit in this disease. © 1998 John Wiley & Sons, Inc. J Biochem Toxicol 13: 93–106, 1999

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From and to the Golgi – defining the Wilson disease protein road map

Polishchuk

2019

FEBS Letters

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Recent studies highlight the continued growth in the identification of a variety of cellular functions that involve the Golgi apparatus. Apart from well‐known membrane sorting/trafficking and glycosylation machineries, the Golgi harbors molecular platforms operating in intracellular signaling, cytoskeleton organization, and protein quality control mechanisms. One of new emerging Golgi functions consists in the regulation of copper homeostasis by coordinating the relocation and activity of copper transporters. Of these, the Cu‐transporting ATPase ATP7B (known as Wilson disease protein) plays a key role in the maintenance of the Cu balance in the body via the supply of essential Cu to the systemic circulation and via elimination of excess Cu into the bile. These activities require tightly regulated shuttling of ATP7B between the Golgi and different post‐Golgi compartments. Despite significant progress over recent years, a number of issues regarding ATP7B trafficking remain to be clarified. This review summarizes current views on ATP7B trafficking pathways from and to the Golgi and underscores the challenges that should be addressed to define the ATP7B trafficking routes and mechanisms in health and disease.

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Biliary copper excretion by hepatocyte lysosomes in the rat. Major excretory pathway in experimental copper overload.

Cited by 107 publications

References 27 publications

Impaired Biliary Excretion of Copper and Lysosomal Enzymes in Long-Evans Cinnamon Rat.

Impaired Biliary Excretion of Copper and Lysosomal Enzymes in Long-Evans Cinnamon Rat.

Molecular mechanisms of copper metabolism and the role of the Menkes disease protein

From and to the Golgi – defining the Wilson disease protein road map

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