Discussion on the relationship between the distribution characteristics of TCM syndrome types and related objective indicators in hepatolenticular degeneration.

Hepatolenticular degeneration is a rare treatable autosomal recessive inherited copper metabolism disorder with a diverse clinical phenotype and an exceptionally complex pathogenesis. Early definitive phenotypic diagnosis and targeted treatment are major challenges worldwide. In this study, we strictly followed the "National Standards of the People's Republic of China - Terminology of Traditional Chinese Medicine Clinical Diagnosis and Treatment (Syndrome Part)," "Chinese Medicine Nomenclature," and the clinical investigation-determined traditional Chinese medicine syndrome differentiation standards at Anhui University of Chinese Medicine to select 6 of the most common traditional Chinese medicine syndrome differentiations.

Glutathione Attenuates Copper Levels and Alleviates Hepatic Injury in TX Mice.

Wilson's disease (WD) is an inherited disorder that is characterized by abnormal copper metabolism, and treatment of this condition in the clinic focuses on promoting copper ion excretion. Glutathione (GSH) is a tripeptide compound whose active group is a sulfhydryl group, which is involved in many important biochemical reactions. Thus, the antioxidant and integrative detoxification effects of GSH have attracted attention.

Metabolomic and biochemical changes in the plasma and liver of toxic milk mice model of Wilson disease.

Wilson disease (WD) is an inherited disorder characterized by abnormal copper metabolism with complex pathological features. Currently, this mechanism of copper overload-induced hepatic injury remains unclear. In this study, male toxic milk (TX) mice were selected as experimental subjects.

Identification and quantitative analysis of the chemical constituents of Gandouling tablets using ultra-high-performance liquid chromatography with quadrupole time-of-flight mass spectrometry.

Gandouling tablets are used in a clinical agent for the treatment of hepatocellular degeneration; however, their chemical constituents have not been elucidated. Here, we screened and identified the chemical constituents of Gandouling tablets using ultra-high-performance liquid chromatography (UHPLC)-quadrupole time of flight/mass spectrometry. A method for the quality evaluation of Gandouling tablets was developed by combining the UHPLC fingerprints and the simultaneous quantitative analysis of multiple active ingredients.

Protective effect of curcumin on hepatolenticular degeneration through copper excretion and inhibition of ferroptosis.

Background: Hepatolenticular degeneration (HLD) is an autosomal recessive disorder concerning copper metabolism. Copper overload is also accompanied by iron overload in HLD patients, which can lead to ferroptosis. Curcumin, the active component in turmeric, has the potential to inhibit ferroptosis.

Protective Mechanism of Gandou Decoction in a Copper-Laden Hepatolenticular Degeneration Model: Pharmacology and Cell Metabolomics.

Gandou decoction (GDD) is a classic prescription for the treatment of hepatolenticular degeneration (HLD) in China; however, the liver-protecting mechanism of this prescription needs further evaluation. In the present study, we explored the protective mechanisms of GDD in a copper-laden HLD model using integrated pharmacology and cellular metabolomics . The results revealed that GDD could significantly promote copper excretion in copper-laden HLD model cells and improve the ultrastructural changes in hepatocytes.

Liver and urine metabolomics reveal the protective effect of Gandou decoction in copper-laden Hepatolenticular degeneration model rats.

Hepatolenticular degeneration (HLD) is an inherited disorder associated with human copper metabolism. Gandou decoction (GDD), a traditional Chinese medicinal formula, has been used as a therapeutic agent for the treatment of HLD in China for decades. Recent pharmacological evaluation in our laboratory has demonstrated that GDD exerts positive and beneficial effects on HLD model rats.

Gandou Decoction Decreases Copper Levels and Alleviates Hepatic Injury in Copper-Laden Hepatolenticular Degeneration Model Rats.

This study was designed to investigate the therapeutic efficacy and underlying mechanisms of Gandou Decoction (GDD) in copper-laden hepatolenticular degeneration (HLD) model rats. In this study, high-performance liquid chromatography (HPLC) fingerprint analysis and eight representative active components were simultaneously measured for quality control of GDD. The therapeutic effect of GDD in HLD was studied by constructing a rat model of copper-laden HLD.

GanDouLing combined with Penicillamine improves cerebrovascular injury via PERK/eIF2α/CHOP endoplasmic reticulum stress pathway in the mouse model of Wilson's disease.

We aim to investigate the function and mechanism of GanDouLing combinated with Penicillamine on cerebrovascular injury in Wilson's disease (WD). ELISA was performed to analyze the expression of vascular injury factors. Pathological changes of cerebral vessels were observed by HE stain.