scholarly journals The function and architecture of DEAH/RHA helicases

2011 ◽  
Vol 2 (4) ◽  
pp. 315-326 ◽  
Author(s):  
Yangzi He ◽  
Gregers R. Andersen ◽  
Klaus H. Nielsen
Keyword(s):  
Translational Regulation ◽  
Acid Metabolism ◽  
Structural Features ◽  
Mrna Splicing ◽  
Innate Immune ◽  
Regulatory Proteins ◽  
Nucleic Acid Metabolism ◽  
Rrna Processing ◽  
Macromolecular Complexes ◽  
Cellular Processes

AbstractHelicases are ubiquitous enzymes that participate in every aspect of nucleic acid metabolism. The DEAH/RHA family of helicases are involved in a variety of cellular processes including transcriptional and translational regulation, pre-mRNA splicing, pre-rRNA processing, mRNA export and decay, in addition to the innate immune response. Recently, the first crystal structures of a DEAH/RHA helicase unveiled the unique structural features of this helicase family. These structures furthermore illuminate the molecular mechanism of these proteins and provide a framework for analysis of their interaction with nucleic acids, regulatory proteins and large macromolecular complexes.

Aicardi-Goutières Syndrome and Systemic Lupus Erythematosus (SLE) in a 12-Year-Old Boy With SAMHD1 Mutations

2011 ◽  
Vol 26 (11) ◽  
pp. 1425-1428 ◽  
Author(s):  
Georgia Ramantani ◽  
Martin Häusler ◽  
Pascal Niggemann ◽  
Britta Wessling ◽  
Hedwig Guttmann ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Acid Metabolism ◽  
Innate Immune ◽  
Nucleic Acid Metabolism ◽  
Interferon Α ◽  
Systemic Lupus ◽  
Sterile Alpha Motif Domain ◽  
Systemic Autoimmunity ◽  
History Of

Aicardi-Goutières syndrome is an early-onset encephalopathy with a presumed immune pathogenesis caused by inherited defects in nucleic acid metabolism. The clinical picture resembles a congenital viral infection despite negative investigations for common viruses. In addition to leukoencephalopathy with calcifications of basal ganglia, patients show increased levels of the antiviral cytokine interferon-α in cerebrospinal fluid. We report on a 12-year-old boy with Aicardi-Goutières syndrome and systemic lupus erythematosus (SLE) due to mutations in the SAMHD1 (sterile alpha motif domain and HD domain-containing protein 1) gene, illustrating an emerging pattern of the natural history of Aicardi-Goutières syndrome characterized by neurological disease followed by symptoms of systemic autoimmunity. Thus, Aicardi-Goutières syndrome constitutes a model disease for systemic autoimmunity triggered by the activation of the innate immune system. Recognition of the etiologic link between Aicardi-Goutières syndrome and systemic lupus erythematosus has direct implications on therapeutic management and suggests that early immune modulatory intervention can improve neurological outcome.

Ribonuclease H2 in health and disease

2014 ◽  
Vol 42 (4) ◽  
pp. 717-725 ◽  
Author(s):  
Martin A.M. Reijns ◽  
Andrew P. Jackson
Keyword(s):  
Nucleic Acid ◽  
Nucleic Acids ◽  
Viral Infection ◽  
Genome Stability ◽  
Innate Immune ◽  
Nucleic Acid Metabolism ◽  
Developmental Studies ◽  
Cellular Processes ◽  
Immune Sensing ◽  
Rnase H2

Innate immune sensing of nucleic acids provides resistance against viral infection and is important in the aetiology of autoimmune diseases. AGS (Aicardi–Goutières syndrome) is a monogenic autoinflammatory disorder mimicking in utero viral infection of the brain. Phenotypically and immunologically, it also exhibits similarities to SLE (systemic lupus erythaematosus). Three of the six genes identified to date encode components of the ribonuclease H2 complex. As all six encode enzymes involved in nucleic acid metabolism, it is thought that pathogenesis involves the accumulation of nucleic acids to stimulate an inappropriate innate immune response. Given that AGS is a monogenic disorder with a defined molecular basis, we use it as a model for common autoimmune disease to investigate cellular processes and molecular pathways responsible for nucleic-acid-mediated autoimmunity. These investigations have also provided fundamental insights into the biological roles of the RNase H2 endonuclease enzyme. In the present article, we describe how human RNase H2 and its role in AGS were first identified, and give an overview of subsequent structural, biochemical, cellular and developmental studies of this enzyme. These investigations have culminated in establishing this enzyme as a key genome-surveillance enzyme required for mammalian genome stability.

EFFECT OF L-CYSTEINE ON α-AMYLASE PRODUCTION AND NUCLEIC ACID METABOLISM IN BACILLUS SUBTILIS

1963 ◽  
Vol 9 (3) ◽  
pp. 337-341 ◽  
Author(s):  
MICHIO OISHI ◽  
SHIGERU KITAYAMA ◽  
HAJIME TAKAHASHI ◽  
BUNJI MARUO
Keyword(s):  
Bacillus Subtilis ◽  
Nucleic Acid ◽  
Acid Metabolism ◽  
Nucleic Acid Metabolism ◽  
Amylase Production

Biochemical studies on the nucleic acid metabolism of embryonic tissue and the effect of drugs

1968 ◽  
Vol 259 (2) ◽  
pp. 186-188 ◽  
Author(s):  
D. Neubert ◽  
E. Oberdisse ◽  
H.-J. Merker ◽  
E. K�hler ◽  
B.-R. Balda
Keyword(s):  
Nucleic Acid ◽  
Acid Metabolism ◽  
Embryonic Tissue ◽  
Nucleic Acid Metabolism ◽  
Biochemical Studies

scholarly journals Enzymes of Nucleic Acid Metabolism from Mung Bean Sprouts

1966 ◽  
Vol 241 (12) ◽  
pp. 2876-2880 ◽  
Author(s):  
Hubert S. Loring ◽  
J.E. McLennan ◽  
Tom L. Walters
Keyword(s):  
Nucleic Acid ◽  
Mung Bean ◽  
Acid Metabolism ◽  
Nucleic Acid Metabolism ◽  
Mung Bean Sprouts ◽  
Bean Sprouts
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