Cysteine metabolism and hydrogen sulfide signaling in Huntington's disease

Bindu D. Paul

doi:10.1016/j.freeradbiomed.2022.05.005

Cysteine metabolism and hydrogen sulfide signaling in Huntington's disease

Bindu D. Paul

School of Medicine

Research output: Contribution to journal › Review article › peer-review

Abstract

The semi-essential amino acid, cysteine, plays important roles in both essential cellular processes as well as in modulation of signaling cascades. Cysteine is obtained both from the diet as well as generated endogenously via the transsulfuration pathway. Cysteine is further utilized in protein synthesis and biosynthesis of various sulfur containing molecules. One of the products of cysteine catabolism, hydrogen sulfide (H₂S), is a gaseous signaling molecule, which regulates a multitude of cellular processes. Cysteine metabolism is dysregulated in several neurodegenerative diseases and during aging. This minireview focuses on aberrant cysteine and H₂S metabolism in Huntington's disease, a neurodegenerative disease caused by expansion of polyglutamine encoding repeats in the gene huntingtin, which leads to motor and cognitive deficits.

Original language	English (US)
Pages (from-to)	93-98
Number of pages	6
Journal	Free Radical Biology and Medicine
Volume	186
DOIs	https://doi.org/10.1016/j.freeradbiomed.2022.05.005
State	Published - Jun 2022

Keywords

Cystathionine γ-lyase
Cysteine
Golgi stress response
Huntington's disease
Hydrogen sulfide
Transsulfuration

ASJC Scopus subject areas

Physiology (medical)
Biochemistry

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10.1016/j.freeradbiomed.2022.05.005

Cite this

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title = "Cysteine metabolism and hydrogen sulfide signaling in Huntington's disease",

abstract = "The semi-essential amino acid, cysteine, plays important roles in both essential cellular processes as well as in modulation of signaling cascades. Cysteine is obtained both from the diet as well as generated endogenously via the transsulfuration pathway. Cysteine is further utilized in protein synthesis and biosynthesis of various sulfur containing molecules. One of the products of cysteine catabolism, hydrogen sulfide (H2S), is a gaseous signaling molecule, which regulates a multitude of cellular processes. Cysteine metabolism is dysregulated in several neurodegenerative diseases and during aging. This minireview focuses on aberrant cysteine and H2S metabolism in Huntington's disease, a neurodegenerative disease caused by expansion of polyglutamine encoding repeats in the gene huntingtin, which leads to motor and cognitive deficits.",

keywords = "Cystathionine γ-lyase, Cysteine, Golgi stress response, Huntington's disease, Hydrogen sulfide, Transsulfuration",

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year = "2022",

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language = "English (US)",

volume = "186",

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T1 - Cysteine metabolism and hydrogen sulfide signaling in Huntington's disease

AU - Paul, Bindu D.

PY - 2022/6

Y1 - 2022/6

N2 - The semi-essential amino acid, cysteine, plays important roles in both essential cellular processes as well as in modulation of signaling cascades. Cysteine is obtained both from the diet as well as generated endogenously via the transsulfuration pathway. Cysteine is further utilized in protein synthesis and biosynthesis of various sulfur containing molecules. One of the products of cysteine catabolism, hydrogen sulfide (H2S), is a gaseous signaling molecule, which regulates a multitude of cellular processes. Cysteine metabolism is dysregulated in several neurodegenerative diseases and during aging. This minireview focuses on aberrant cysteine and H2S metabolism in Huntington's disease, a neurodegenerative disease caused by expansion of polyglutamine encoding repeats in the gene huntingtin, which leads to motor and cognitive deficits.

AB - The semi-essential amino acid, cysteine, plays important roles in both essential cellular processes as well as in modulation of signaling cascades. Cysteine is obtained both from the diet as well as generated endogenously via the transsulfuration pathway. Cysteine is further utilized in protein synthesis and biosynthesis of various sulfur containing molecules. One of the products of cysteine catabolism, hydrogen sulfide (H2S), is a gaseous signaling molecule, which regulates a multitude of cellular processes. Cysteine metabolism is dysregulated in several neurodegenerative diseases and during aging. This minireview focuses on aberrant cysteine and H2S metabolism in Huntington's disease, a neurodegenerative disease caused by expansion of polyglutamine encoding repeats in the gene huntingtin, which leads to motor and cognitive deficits.

KW - Cystathionine γ-lyase

KW - Cysteine

KW - Golgi stress response

KW - Huntington's disease

KW - Hydrogen sulfide

KW - Transsulfuration

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DO - 10.1016/j.freeradbiomed.2022.05.005

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AN - SCOPUS:85129965565

SN - 0891-5849

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JO - Free Radical Biology and Medicine

JF - Free Radical Biology and Medicine

ER -

Cysteine metabolism and hydrogen sulfide signaling in Huntington's disease

Abstract

Keywords

ASJC Scopus subject areas

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