Copper Homeostasis: Specialized Functions of the Late Secretory Pathway

Gitlin, Jonathan D.

doi:10.1016/j.devcel.2014.06.002

Cited by 5 publications

(4 citation statements)

References 8 publications

(9 reference statements)

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“…We further investigated the copper-removing efficiency of MB at the whole-organ level (Figure 3, G-I, Supplemental Figure 3). During a 2-hour perfusion of Atp7b -/-livers, MB released 10-fold greater amounts of copper into bile than did TTM ( Figure 3G and Supplemental Figure 3B), the major physiological copper excretion route (36). D-PA and TETA did not provoke any detectable release of copper into bile ( Figure 3G).…”

Section: Increasing Copper Load Impairs Mitochondrial Membrane Integrmentioning

confidence: 99%

Methanobactin reverses acute liver failure in a rat model of Wilson disease

Lichtmannegger¹,

Leitzinger²,

Wimmer³

et al. 2016

Journal of Clinical Investigation

133

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Section: Increasing Copper Load Impairs Mitochondrial Membrane Integrmentioning

confidence: 99%

Methanobactin reverses acute liver failure in a rat model of Wilson disease

Lichtmannegger¹,

Leitzinger²,

Wimmer³

et al. 2016

Journal of Clinical Investigation

133

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“…Copper i ons (Cu 2+ ) a re requi red as an ess ential cofa ctor i n numerous redox enz ymes that are invol ved in vari ous cri tical physi ol ogi cal process es, s uch as cell ular res pirati on, iron homeostasi s, pi gmentati on, neurotra ns mi tter s ynthesis a nd metabolis m, pepti de bi ogenesis, a ntioxi da nt def ense a nd connecti ve tis sue formati on 1,2 . Copper i ons (Cu 2+ ) a re requi red as an ess ential cofa ctor i n numerous redox enz ymes that are invol ved in vari ous cri tical physi ol ogi cal process es, s uch as cell ular res pirati on, iron homeostasi s, pi gmentati on, neurotra ns mi tter s ynthesis a nd metabolis m, pepti de bi ogenesis, a ntioxi da nt def ense a nd connecti ve tis sue formati on 1,2 .…”

Section: Introductionmentioning

confidence: 99%

“…Copper ions (Cu 2+ ) are required as an essential cofactor in numerous redox enzymes that are involved in various critical physiological processes, such as cellular respiration, iron homeostasis, pigmentation, neurotransmitter synthesis and metabolism, peptide biogenesis, antioxidant defense and connective tissue formation. 1,2 On the other hand, the disturbance of copper homeostasis in cells is related to numerous severe disorders such as Menkes syndrome, 3,4 Wilson's disease, [5][6][7] Alzheimer's disease 8,9 and familial amyotrophic lateral sclerosis. 10,11 Therefore, it is vital to maintain the subtle copper homoeostasis for the health of human beings.…”

Section: Introductionmentioning

confidence: 99%

A six-membered-ring incorporated Si-rhodamine for imaging of copper(ii) in lysosomes

et al. 2016

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The regulation of copper homeostasis in lysosomes of living cells is closely related to various physiological and pathological processes. Thus, it is of urgent need to develop a fluorescent probe for selectively and sensitively monitoring the location and concentration of lysosomal Cu(2+). Herein, a six-membered ring, thiosemicarbazide, was incorporated into a Si-rhodamine (SiR) scaffold for the first time, affording a SiR-based fluorescent probe SiRB-Cu. Through the effective Cu(2+)-triggered ring-opening process, the probe exhibits fast NIR chromogenic and fluorogenic responses to Cu(2+) within 2 min as the result of formation of a highly fluorescent product SiR-NCS. Compared with a five-membered ring, the expanded ring retains great tolerance to H(+), ensuring the superior sensitivity with a detection limit as low as 7.7 nM and 200-fold enhancement of relative fluorescence in the presence of 1.0 equiv. of Cu(2+) in pH = 5.0 solution, the physiological pH of lysosome. Moreover, the thiosemicarbazide moiety acts not only as the chelating and reactive site, but also as an efficient lysosome-targeting group, leading to the proactive accumulation of the probe into lysosomes. Taking advantage of these distinct properties, SiRB-Cu provides a functional probe suitable for imaging exogenous and endogenous lysosomal Cu(2+) with high imaging contrast and fidelity.

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“…Binding of the copper to ATP7B provokes ATP hydrolysis, producing energy for copper transport from the cytosol to Golgi lumen [ 30 ]. More recent studies have indicated that ATP7B is redistributed from TGN to the late endosome/lysosome with the increase of copper concentration in hepatocytes [ 31 , 32 ].…”

Section: Function and Regulation Of Atp7b In Copper Transportationmentioning

confidence: 99%

Wilson’s Disease: A Comprehensive Review of the Molecular Mechanisms

Wang

et al. 2015

IJMS

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Wilson’s disease (WD), also known as hepatolenticular degeneration, is an autosomal recessive inherited disorder resulting from abnormal copper metabolism. Reduced copper excretion causes an excessive deposition of the copper in many organs such as the liver, central nervous system (CNS), cornea, kidney, joints, and cardiac muscle where the physiological functions of the affected organs are impaired. The underlying molecular mechanisms for WD have been extensively studied. It is now believed that a defect in P-type adenosine triphosphatase (ATP7B), the gene encoding the copper transporting P-type ATPase, is responsible for hepatic copper accumulation. Deposited copper in the liver produces toxic effects via modulating several molecular pathways. WD can be a lethal disease if left untreated. A better understanding of the molecular mechanisms causing the aberrant copper deposition and organ damage is the key to developing effective management approaches.

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Copper Homeostasis: Specialized Functions of the Late Secretory Pathway

Cited by 5 publications

References 8 publications

Methanobactin reverses acute liver failure in a rat model of Wilson disease

Methanobactin reverses acute liver failure in a rat model of Wilson disease

A six-membered-ring incorporated Si-rhodamine for imaging of copper(ii) in lysosomes

Wilson’s Disease: A Comprehensive Review of the Molecular Mechanisms

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