Role of endoplasmic reticulum stress and protein misfolding in disorders of the liver and pancreas

J Lukas, J Pospech, C Oppermann, C Hund… - Advances in medical …, 2019 - Elsevier
The endoplasmic reticulum (ER) is the site of synthesis and folding of membrane and
secretory proteins. The fraction of protein passing through the ER represents a large …

ACVIM consensus statement on the diagnosis and treatment of chronic hepatitis in dogs

CRL Webster, SA Center, JM Cullen… - Journal of veterinary …, 2019 - Wiley Online Library
This consensus statement on chronic hepatitis (CH) in dogs is based on the expert opinion
of 7 specialists with extensive experience in diagnosing, treating, and conducting clinical …

Regulation of the apico-basolateral trafficking polarity of the homologous copper-ATPases ATP7A and ATP7B

Ruturaj, M Mishra, S Saha, S Maji… - Journal of Cell …, 2024 - journals.biologists.com
The homologous P-type copper-ATPases (Cu-ATPases) ATP7A and ATP7B are the key
regulators of copper homeostasis in mammalian cells. In polarized epithelia, upon copper …

Polarized trafficking and copper transport activity of ATP7B: A mutational approach to establish genotype–phenotype correlation in Wilson disease

S Das, A Mohammed, T Mandal, S Maji… - Human …, 2022 - Wiley Online Library
Mutation in ATP7B gene causes Wilson disease (WD) that is characterized by severe
hepatic and neurological symptoms. ATP7B localizes at the trans‐Golgi Network (TGN) …

ATP7B Binds Ruthenium(II) p-Cymene Half-Sandwich Complexes: Role of Steric Hindrance and Ru–I Coordination in Rescuing the Sequestration

K Purkait, Ruturaj, A Mukherjee, A Gupta - Inorganic chemistry, 2019 - ACS Publications
Ruthenium (II/III) complexes are predicted to be efficient alternatives to platinum drug-
resistant cancers but have never been investigated for sequestration and efflux by Cu …

Retromer retrieves the Wilson disease protein ATP7B from endolysosomes in a copper-dependent manner

S Das, S Maji, Ruturaj, I Bhattacharya… - Journal of cell …, 2020 - journals.biologists.com
The Wilson disease protein, ATP7B maintains copper (herein referring to the Cu+ ion)
homeostasis in the liver. ATP7B traffics from trans-Golgi network to endolysosomes to export …

Long-term correction of copper metabolism in Wilson's disease mice with AAV8 vector delivering truncated ATP7B

Y Leng, P Li, L Zhou, L Xiao, Y Liu, Z Zheng… - Human gene …, 2019 - liebertpub.com
Wilson's disease (WD) is an autosomal recessive disorder of copper metabolism caused by
mutations in the ATP7B gene encoding a liver active copper transport enzyme. Gene …

Characterization of a fluorescent 1,8-naphthalimide-functionalized PAMAM dendrimer and its Cu(ii) complexes as cytotoxic drugs: EPR and biological studies in …

B Canonico, M Cangiotti, M Montanari, S Papa… - Biological …, 2022 - degruyter.com
The activity and interacting ability of a polyamidoamine (PAMAM) dendrimer modified with 4-
N-methylpiperazine-1, 8-naphthalimide units (termed D) and complexed by Cu (ii) ions …

Wilson disease–causing mutations in the carboxyl terminus of ATP7B regulates its localization and Golgi exit selectively in the unpolarized cells

K Chakraborty, S Das, A Pal, S Maji, B Rai… - Metallomics, 2023 - academic.oup.com
Mutational inactivation of the P-type Cu-ATPase ATP7B interferes with its cellular functions
to varying extent leading to varied cellular phenotypes. Wilson's disease (WD) primarily …

Computing the pathogenicity of Wilson's disease ATP7B mutations: implications for disease prevalence

N Tang, TD Sandahl, P Ott, KP Kepp - Journal of Chemical …, 2019 - ACS Publications
Genetic variations in the gene encoding the copper-transport protein ATP7B are the primary
cause of Wilson's disease. Controversially, clinical prevalence seems much smaller than the …