Abstract:
:Several genes have been identified for monogenic disorders that variably resemble Parkinson's disease. Dominant mutations in the gene encoding alpha-synuclein enhance the propensity of this protein to aggregate. As a consequence, these patients have a widespread disease with protein inclusion bodies in several brain areas. In contrast, mutations in several recessive genes (parkin, DJ-1, and PINK1) produce neuronal cell loss but generally without protein aggregation pathology. Progress has been made in understanding some of the mechanisms of toxicity: Parkin is an E3 ubiquitin ligase and DJ-1 and PINK1 appear to protect against mitochondrial damage. However, we have not yet fully resolved how the recessive genes relate to alpha-synuclein, or whether they represent different ways to induce a similar phenotype.
journal_name
Annu Rev Biochemjournal_title
Annual review of biochemistryauthors
Cookson MRdoi
10.1146/annurev.biochem.74.082803.133400subject
Has Abstractpub_date
2005-01-01 00:00:00pages
29-52eissn
0066-4154issn
1545-4509journal_volume
74pub_type
杂志文章,评审abstract::DNA looping is widely used in nature. It is well documented in the regulation of prokaryotic and eukaryotic gene expression, DNA replication, and site-specific DNA recombination. Undoubtedly looping also functions in other protein-DNA transactions such as repair and chromosome segregation. While the underlying physica...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.bi.61.070192.001215
更新日期:1992-01-01 00:00:00
abstract::The existence and function of actin in the nucleus has been hotly debated for forty years. Recently, beta-actin was found to be a component of mammalian SWI/SNF-like BAF chromatin remodeling complexes and still more recently other SWI/SNF-related chromatin remodeling complexes in yeast, flies, and man. Although the fu...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.71.110601.135507
更新日期:2002-01-01 00:00:00
abstract::This review is concerned with the structures and mechanisms of a superfamily of regulatory GTP hydrolases (G proteins). G proteins include Ras and its close homologs, translation elongation factors, and heterotrimeric G proteins. These proteins share a common structural core, exemplified by that of p21ras (Ras), and s...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.66.1.639
更新日期:1997-01-01 00:00:00
abstract::Many critical biological processes take place at hydrophobic:hydrophilic interfaces, and a wide range of organisms produce surface-active proteins and peptides that reduce surface and interfacial tension and mediate growth and development at these boundaries. Microorganisms produce both small lipid-associated peptides...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-061516-044847
更新日期:2017-06-20 00:00:00
abstract::Clathrin-mediated endocytosis (CME) is the major endocytic pathway in mammalian cells. It is responsible for the uptake of transmembrane receptors and transporters, for remodeling plasma membrane composition in response to environmental changes, and for regulating cell surface signaling. CME occurs via the assembly an...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-062917-012644
更新日期:2018-06-20 00:00:00
abstract::The determination of several structures of nuclear receptor ligand binding domains (LBD) has led to new insights into the mechanism of action of this very important class of receptors. This review describes and compares the different LBD structures and their relationship to the function of the nuclear receptors. The r...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.68.1.559
更新日期:1999-01-01 00:00:00
abstract::The structure and integrity of telomeres are essential for genome stability. Telomere dysregulation can lead to cell death, cell senescence, or abnormal cell proliferation. The maintenance of telomere repeats in most eukaryotic organisms requires telomerase, which consists of a reverse transcriptase (RT) and an RNA te...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.75.103004.142412
更新日期:2006-01-01 00:00:00
abstract::Glycoproteins have a wide distribution in nature and serve a vast number of functions. There are glycoprotein enzymes and hormones; glycoproteins are found in blood and secretions, in cell membranes, and in connective tissue. Of all the biologically occurring macromolecules the glycoproteins, which consist of carbohyd...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.bi.45.070176.001245
更新日期:1976-01-01 00:00:00
abstract::The past few years have seen exciting advances in understanding the structure and function of catalytic RNA. Crystal structures of several ribozymes have provided detailed insight into the folds of RNA molecules. Models of other biologically important RNAs have been constructed based on structural, phylogenetic, and b...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.69.1.597
更新日期:2000-01-01 00:00:00
abstract::The maintenance of the eukaryotic genome requires precisely coordinated replication of the entire genome each time a cell divides. To achieve this coordination, eukaryotic cells use an ordered series of steps to form several key protein assemblies at origins of replication. Recent studies have identified many of the p...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.71.110601.135425
更新日期:2002-01-01 00:00:00
abstract::The nanoscale engineering of nucleic acids has led to exciting molecular technologies for high-end biological imaging. The predictable base pairing, high programmability, and superior new chemical and biological methods used to access nucleic acids with diverse lengths and in high purity, coupled with computational to...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-060815-014244
更新日期:2016-06-02 00:00:00
abstract::Staphylococcus aureus is a major human and veterinary pathogen worldwide. Methicillin-resistant S. aureus (MRSA) poses a significant and enduring problem to the treatment of infection by such strains. Resistance is usually conferred by the acquisition of a nonnative gene encoding a penicillin-binding protein (PBP2a), ...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-060614-034516
更新日期:2015-01-01 00:00:00
abstract::Proteins are increasingly used in basic and applied biomedical research. Many proteins, however, are only marginally stable and can be expressed in limited amounts, thus hampering research and applications. Research has revealed the thermodynamic, cellular, and evolutionary principles and mechanisms that underlie marg...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-062917-012102
更新日期:2018-06-20 00:00:00
abstract::This review focuses on molecular mechanisms that underlie the communication between the nuclear and mitochondrial genomes in eukaryotic cells. These genomes interact in at least two ways. First, they contribute essential subunit polypeptides to important mitochondrial proteins; second, they collaborate in the synthesi...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.bi.65.070196.003023
更新日期:1996-01-01 00:00:00
abstract::G protein-coupled receptor kinases (GRKs) constitute a family of six mammalian serine/threonine protein kinases that phosphorylate agonist-bound, or activated, G protein-coupled receptors (GPCRs) as their primary substrates. GRK-mediated receptor phosphorylation rapidly initiates profound impairment of receptor signal...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.67.1.653
更新日期:1998-01-01 00:00:00
abstract::Phylum Apicomplexa comprises a large group of obligate intracellular parasites of high medical and veterinary importance. These organisms succeed intracellularly by effecting remarkable changes in a broad range of diverse host cells. The transformation of the host erythrocyte is particularly striking in the case of th...
journal_title:Annual review of biochemistry
pub_type: 杂志文章
doi:10.1146/annurev-biochem-060614-034157
更新日期:2015-01-01 00:00:00
abstract::The protein kinase Akt/PKB is activated via a multistep process by a variety of signals. In the early steps of this process, PI-3 kinase-generated D3-phosphorylated phosphoinositides bind the Akt PH domain and induce the translocation of the kinase to the plasma membrane where it co-localizes with phosphoinositide-dep...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.68.1.965
更新日期:1999-01-01 00:00:00
abstract::Proton transfer in the bacterial RC associated with the reduction of the bound QB to the dihydroquinone is an important step in the energetics of photosynthetic bacteria. The binding of two protons by the quinone is associated with the transfer of the second electron to QB at a rate of ca. 10(3) s-1 (pH 7). Mutation o...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.bi.61.070192.004241
更新日期:1992-01-01 00:00:00
abstract::A childhood fascination with animals, plants, and insects was aided and abetted by many giants, beginning with my parents. The Bronx High School of Science and the City College of New York (CCNY) made a solid and priceless grounding in chemistry and biology available free of charge. Abe Mazur at CCNY revealed the wond...
journal_title:Annual review of biochemistry
pub_type: 传,历史文章,杂志文章
doi:10.1146/annurev.biochem.72.081902.140918
更新日期:2003-01-01 00:00:00
abstract::The bacterial TolC protein plays a common role in the expulsion of diverse molecules, which include protein toxins and antibacterial drugs, from the cell. TolC is a trimeric 12-stranded alpha/beta barrel, comprising an alpha-helical trans-periplasmic tunnel embedded in the outer membrane by a contiguous beta-barrel ch...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.73.011303.074104
更新日期:2004-01-01 00:00:00
abstract::The majority of cellular energy in the form of adenosine triphosphate (ATP) is synthesized by the ubiquitous F(1)F(0) ATP synthase. Power for ATP synthesis derives from an electrochemical proton (or Na(+)) gradient, which drives rotation of membranous F(0) motor components. Efficient rotation not only requires a signi...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.78.081307.104803
更新日期:2009-01-01 00:00:00
abstract::The assembly of individual proteins into functional complexes is fundamental to nearly all biological processes. In recent decades, many thousands of homomeric and heteromeric protein complex structures have been determined, greatly improving our understanding of the fundamental principles that control symmetric and a...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-060614-034142
更新日期:2015-01-01 00:00:00
abstract::The lipid A moiety of lipopolysaccharide forms the outer monolayer of the outer membrane of most gram-negative bacteria. Escherichia coli lipid A is synthesized on the cytoplasmic surface of the inner membrane by a conserved pathway of nine constitutive enzymes. Following attachment of the core oligosaccharide, nascen...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.76.010307.145803
更新日期:2007-01-01 00:00:00
abstract::Mechanisms of chemical reaction can often be predicted by determining the dependence of the lifetime of reaction intermediates on the structure of the reactants. When there is no lifetime in the presence of another reactant or catalyst the reaction proceeds through an enforced concerted mechanism. Noncovalent binding ...
journal_title:Annual review of biochemistry
pub_type: 传,历史文章,杂志文章
doi:10.1146/annurev.biochem.66.1.1
更新日期:1997-01-01 00:00:00
abstract::Microtubules are polymers that are essential for, among other functions, cell transport and cell division in all eukaryotes. The regulation of the microtubule system includes transcription of different tubulin isotypes, folding of /¿-tubulin heterodimers, post-translation modification of tubulin, and nucleotide-based ...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.69.1.277
更新日期:2000-01-01 00:00:00
abstract::Sorting of transmembrane proteins to endosomes and lysosomes is mediated by signals present within the cytosolic domains of the proteins. Most signals consist of short, linear sequences of amino acid residues. Some signals are referred to as tyrosine-based sorting signals and conform to the NPXY or YXXO consensus moti...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.72.121801.161800
更新日期:2003-01-01 00:00:00
abstract::The repair of DNA by homologous recombination is an essential, efficient, and high-fidelity process that mends DNA lesions formed during cellular metabolism; these lesions include double-stranded DNA breaks, daughter-strand gaps, and DNA cross-links. Genetic defects in the homologous recombination pathway undermine ge...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-060614-034352
更新日期:2016-06-02 00:00:00
abstract::Numerous membrane proteins form multisubunit protein complexes, which contain both integral and peripheral subunits, in addition to prosthetic groups. Bacterial membrane proteins are inserted into the inner membrane by the Sec translocase and YidC insertase. Their folding can be facilitated by YidC and the phospholipi...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev-biochem-060409-092524
更新日期:2011-01-01 00:00:00
abstract::The rhodopsin crystal structure provides a structural basis for understanding the function of this and other G protein-coupled receptors (GPCRs). The major structural motifs observed for rhodopsin are expected to carry over to other GPCRs, and the mechanism of transformation of the receptor from inactive to active for...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.biochem.75.103004.142743
更新日期:2006-01-01 00:00:00
abstract::The T lymphocytes of the vertebrate immune system look for changes that take place within the organism by examining a display of peptides at the cell surface. These peptides are presented by the products of the major histocompatibility complex (MHC). MHC class I products present peptides derived by proteolysis of cyto...
journal_title:Annual review of biochemistry
pub_type: 杂志文章,评审
doi:10.1146/annurev.bi.64.070195.002335
更新日期:1995-01-01 00:00:00