NDLI: Molecular basis for dysfunction of some mutant forms of methylmalonyl-CoA mutase: deductions from the structure of methionine synthase.

Please wait, while we are loading the content...

Year: 2017 Volume: 114

Year: 2016 Volume: 113

Year: 2015 Volume: 112

Year: 2014 Volume: 111

Year: 2013 Volume: 110

Year: 2012 Volume: 109

Year: 2011 Volume: 108

Year: 2010 Volume: 107

Year: 2009 Volume: 106

Year: 2008 Volume: 105

Year: 2007 Volume: 104

Year: 2006 Volume: 103

Year: 2005 Volume: 102

Year: 2004 Volume: 101

Year: 2003 Volume: 100

Year: 2002 Volume: 99

Year: 2001 Volume: 98

Year: 2000 Volume: 97

Year: 1999 Volume: 96

Year: 1998 Volume: 95

Year: 1997 Volume: 94

Year: 1996 Volume: 93

Year: 1996 Volume: 93 Issue: 26

Year: 1996 Volume: 93 Issue: 25

Year: 1996 Volume: 93 Issue: 24

Year: 1996 Volume: 93 Issue: 23

Year: 1996 Volume: 93 Issue: 22

Year: 1996 Volume: 93 Issue: 21

Year: 1996 Volume: 93 Issue: 20

Year: 1996 Volume: 93 Issue: 19

Year: 1996 Volume: 93 Issue: 18

Year: 1996 Volume: 93 Issue: 17

Year: 1996 Volume: 93 Issue: 16

Year: 1996 Volume: 93 Issue: 15

Year: 1996 Volume: 93 Issue: 14

Year: 1996 Volume: 93 Issue: 13

Year: 1996 Volume: 93 Issue: 12

Year: 1996 Volume: 93 Issue: 11

Successful external fertilization in turbulent environments.

A mutation that allows endosperm development without fertilization.

Congruence of fatty acid methyl ester profiles and morphological characters of arbuscular mycorrhizal fungi in Gigasporaceae.

Diverse strategies for tetracycline-regulated inducible gene expression.

The viruses in all of us: characteristics and biological significance of human endogenous retrovirus sequences.

Rapid retroviral delivery of tetracycline-inducible genes in a single autoregulatory cassette.

Crystal structure at 2.6-A resolution of human macrophage migration inhibitory factor.

Cloning and expression in Escherichia coli of the OGG1 gene of Saccharomyces cerevisiae, which codes for a DNA glycosylase that excises 7,8-dihydro-8-oxoguanine and 2,6-diamino-4-hydroxy-5-N-methylformamidopyrimidine.

Quantal acetylcholine release induced by mediatophore transfection.

Absolute mRNA levels and transcriptional initiation rates in Saccharomyces cerevisiae.

Immunotargeting of antioxidant enzyme to the pulmonary endothelium.

The rat extracellular superoxide dismutase dimer is converted to a tetramer by the exchange of a single amino acid.

A structural model for a homeotic protein-extradenticle-DNA complex accounts for the choice of HOX protein in the heterodimer.

Malignant conversion of chemically transformed normal human cells.

Traumatic brain damage prevented by the non-N-methyl-D-aspartate antagonist 2,3-dihydroxy-6-nitro-7-sulfamoylbenzo[f] quinoxaline.

Arachidonate lipoxygenases as essential regulators of cell survival and apoptosis.

Long-term culture of lymphohematopoietic stem cells.

Expression of a recoded nuclear gene inserted into yeast mitochondrial DNA is limited by mRNA-specific translational activation.

Ultraviolet-B photodestruction of a light-harvesting complex.

Functional testicular tissue does not masculinize development of the zebra finch song system.

Inhibition of immunoglobulin folding and secretion by dominant negative BiP ATPase mutants.

Evidence for a role for the phosphotyrosine-binding domain of Shc in interleukin 2 signaling.

Cloning of thermostable DNA polymerases from hyperthermophilic marine Archaea with emphasis on Thermococcus sp. 9 degrees N-7 and mutations affecting 3'-5' exonuclease activity.

Cloning of rat MEK kinase 1 cDNA reveals an endogenous membrane-associated 195-kDa protein with a large regulatory domain.

Late Holocene human-induced modifications to a central Polynesian island ecosystem.

Heat-shock protein 104 expression is sufficient for thermotolerance in yeast.

Adenine phosphoribosyltransferase-deficient mice develop 2,8-dihydroxyadenine nephrolithiasis.

The transcription factors c-myb and GATA-2 act independently in the regulation of normal hematopoiesis.

Bcl-2 interrupts the ceramide-mediated pathway of cell death.

NMR studies of muscle glycogen synthesis in insulin-resistant offspring of parents with non-insulin-dependent diabetes mellitus immediately after glycogen-depleting exercise.

Expression of Norwalk virus capsid protein in transgenic tobacco and potato and its oral immunogenicity in mice.

Direct observation of iron-induced conformational changes of mitochondrial DNA by high-resolution field-emission in-lens scanning electron microscopy.

Expression of a renal type I sodium/phosphate transporter (NaPi-1) induces a conductance in Xenopus oocytes permeable for organic and inorganic anions.

Ras2 signals via the Cdc42/Ste20/mitogen-activated protein kinase module to induce filamentous growth in Saccharomyces cerevisiae.

Three-dimensional structure of human protein kinase C interacting protein 1, a member of the HIT family of proteins.

Insights into antibody catalysis: structure of an oxygenation catalyst at 1.9-angstrom resolution.

Near-membrane [Ca2+] transients resolved using the Ca2+ indicator FFP18.

Disruption of the aldolase A tetramer into catalytically active monomers.

Ancestral major histocompatibility complex DRB genes beget conserved patterns of localized polymorphisms.

Phenotypic variations among paternal centrosomes expressed within the zygote as disparate microtubule lengths and sperm aster organization: correlations between centrosome activity and developmental success.

Vip3A, a novel Bacillus thuringiensis vegetative insecticidal protein with a wide spectrum of activities against lepidopteran insects.

Extremely sensitive, background-free gene detection using binary probes and beta replicase.

Molecular cloning and expression of a cyclic AMP-activated chloride conductance regulator: a novel ATP-binding cassette transporter.

Mechanism of photoreceptor cGMP phosphodiesterase inhibition by its gamma-subunits.

Intrasarcomere [Ca2+] gradients in ventricular myocytes revealed by high speed digital imaging microscopy.

Recombinational repair of gaps in DNA is asymmetric in Ustilago maydis and can be explained by a migrating D-loop model.

Differential effects of staphylococcal toxic shock syndrome toxin-1 on B cell apoptosis.

Targeted disruption of the mZP3 gene results in production of eggs lacking a zona pellucida and infertility in female mice.

Purification and cDNA cloning of a second apoptosis-related cysteine protease that cleaves and activates sterol regulatory element binding proteins.

Conserved motifs in prokaryotic and eukaryotic polypeptide release factors: tRNA-protein mimicry hypothesis.

Evolutionary relationships of the coelacanth, lungfishes, and tetrapods based on the 28S ribosomal RNA gene.

Mechanism for transcriptional gain of function resulting from chromosomal translocation in alveolar rhabdomyosarcoma.

Irreversible inactivation of interleukin 2 in a pump-based delivery environment.

The multidomain protein Trio binds the LAR transmembrane tyrosine phosphatase, contains a protein kinase domain, and has separate rac-specific and rho-specific guanine nucleotide exchange factor domains.

The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease.

Ca2+ channel blockers modulate metabolism of collagens within the extracellular matrix.

A locus on chromosome 7 determines myocardial cell necrosis and calcification (dystrophic cardiac calcinosis) in mice.

Subcellular imaging of intramitochondrial Ca2+ with recombinant targeted aequorin: significance for the regulation of pyruvate dehydrogenase activity.

Cellular and subcellular localization of the vasopressin- regulated urea transporter in rat kidney.

Host-parasite dynamics and outgrowth of virus containing a single K70R amino acid change in reverse transcriptase are responsible for the loss of human immunodeficiency virus type 1 RNA load suppression by zidovudine.

The adipocyte specific transcription factor C/EBPalpha modulates human ob gene expression.

The protection receptor for IgG catabolism is the beta2-microglobulin-containing neonatal intestinal transport receptor.

Cloning and characterization of a specific coactivator, ARA70, for the androgen receptor in human prostate cells.

The replication initiator protein pi of the plasmid R6K specifically interacts with the host-encoded helicase DnaB.

Regulation of cardiac sodium-calcium exchanger by beta-adrenergic agonists.

Apoptosis of nur77/N10-transgenic thymocytes involves the Fas/Fas ligand pathway.

Dopamine transporters are markedly reduced in Lesch-Nyhan disease in vivo.

Interaction of cyclooxygenases with an apoptosis- and autoimmunity-associated protein.

Molecular basis for dysfunction of some mutant forms of methylmalonyl-CoA mutase: deductions from the structure of methionine synthase.

Microfabricated structures for integrated DNA analysis.

Ordered yeast artificial chromosome clones representing the Dictyostelium discoideum genome.

The timing of synaptic vesicle endocytosis.

A loop-loop 'kissing' complex is the essential part of the dimer linkage of genomic HIV-1 RNA.

Activation of the translational suppressor 4E-BP1 following infection with encephalomyocarditis virus and poliovirus.

Glutamate receptor blockade at cortical synapses disrupts development of thalamocortical and columnar organization in somatosensory cortex.

Active barnase variants with completely random hydrophobic cores.

Pea formaldehyde-active class III alcohol dehydrogenase: common derivation of the plant and animal forms but not of the corresponding ethanol-active forms (classes I and P).

Vitamin E prevents oxidative modification of brain and lymphocyte band 3 proteins during aging.

Nitric oxide inhibits creatine kinase and regulates rat heart contractile reserve.

Two mutant prion proteins expressed in cultured cells acquire biochemical properties reminiscent of the scrapie isoform.

Direct interaction of the Wiskott-Aldrich syndrome protein with the GTPase Cdc42.

Two mitochondrial group I introns in a metazoan, the sea anemone Metridium senile: one intron contains genes for subunits 1 and 3 of NADH dehydrogenase.

A novel iron-regulated metal transporter from plants identified by functional expression in yeast.

Cellular stress inhibits transposition of the yeast retrovirus-like element Ty3 by a ubiquitin-dependent block of virus-like particle formation.

The contrasting roles of ICE family proteases and interleukin-1beta in apoptosis induced by trophic factor withdrawal and by copper/zinc superoxide dismutase down-regulation.

A functional peptide encoded in the Escherichia coli 23S rRNA.

A truncated mutant (residues 58-140) of the hepatitis B virus X protein retains transactivation function.

Characterization of a unique variant of bat rabies virus responsible for newly emerging human cases in North America.

Constitutively active human Notch1 binds to the transcription factor CBF1 and stimulates transcription through a promoter containing a CBF1-responsive element.

OB protein binds specifically to the choroid plexus of mice and rats.

Conserved catalytic machinery and the prediction of a common fold for several families of glycosyl hydrolases. (Journal: Proceedings of the National Academy of Sciences of the United States of America Volume: 93 Issue: 11)

Year: 1996 Volume: 93 Issue: 10

Year: 1996 Volume: 93 Issue: 9

Year: 1996 Volume: 93 Issue: 8

Year: 1996 Volume: 93 Issue: 7

Year: 1996 Volume: 93 Issue: 6

Year: 1996 Volume: 93 Issue: 5

Year: 1996 Volume: 93 Issue: 4

Year: 1996 Volume: 93 Issue: 3

Year: 1996 Volume: 93 Issue: 2

Year: 1996 Volume: 93 Issue: 1

Year: 1995 Volume: 92

Year: 1994 Volume: 91

Year: 1993 Volume: 90

Year: 1992 Volume: 89

Year: 1991 Volume: 88

Year: 1990 Volume: 87

Year: 1989 Volume: 86

Year: 1988 Volume: 85

Year: 1987 Volume: 84

Year: 1986 Volume: 83

Year: 1985 Volume: 82

Year: 1984 Volume: 81

Year: 1983 Volume: 80

Year: 1982 Volume: 79

Year: 1981 Volume: 78

Year: 1980 Volume: 77

Year: 1979 Volume: 76

Year: 1978 Volume: 75

Year: 1977 Volume: 74

Year: 1976 Volume: 73

Year: 1975 Volume: 72

Year: 1974 Volume: 71

Year: 1973 Volume: 70

Year: 1972 Volume: 69

Year: 1971 Volume: 68

Year: 1970 Volume: 67

Year: 1970 Volume: 66

Year: 1970 Volume: 65

Year: 1969 Volume: 64

Year: 1969 Volume: 63

Year: 1969 Volume: 62

Year: 1968 Volume: 61

Year: 1968 Volume: 60

Year: 1968 Volume: 59

Year: 1967 Volume: 58

Year: 1967 Volume: 57

Year: 1966 Volume: 56

Year: 1966 Volume: 55

Molecular basis for dysfunction of some mutant forms of methylmalonyl-CoA mutase: deductions from the structure of methionine synthase.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Drennan, C. L. Ledley, F. D. Ludwig, M. L. Fenton, W. A. Matthews, R. G. Rosenblatt, D. S.
Description	Author Affiliation: Drennan CL ( Biophysics Research Division and Department of Biological Chemistry, The University of Michigan, Ann Arbor 48109, USA.);
Abstract	Inherited defects in the gene for methylmalonyl-CoA mutase (EC 5.4.99.2) result in the mut forms of methylmalonic aciduria. mut- mutations lead to the absence of detectable mutase activity and are not corrected by excess cobalamin, whereas mut- mutations exhibit residual activity when exposed to excess cobalamin. Many of the mutations that cause methylmalonic aciduria in humans affect residues in the C-terminal region of the methylmalonyl-CoA mutase. This portion of the methylmalonyl-CoA mutase sequence can be aligned with regions in other B12 (cobalamin)-dependent enzymes, including the C-terminal portion of the cobalamin-binding region of methionine synthase. The alignments allow the mutations of human methylmalonyl-CoA mutase to be mapped onto the structure of the cobalamin-binding fragment of methionine synthase from Escherichia coli (EC 2.1.1.13), which has recently been determined by x-ray crystallography. In this structure, the dimethylbenzimidazole ligand to the cobalt in free cobalamin has been displaced by a histidine ligand, and the dimethylbenzimidazole nucleotide 'tail' is thrust into a deep hydrophobic pocket in the protein. Previously identified mut0 and mut- mutations (Gly-623 --> Arg, Gly-626 --> Cys, and Gly-648 --> Asp) of the mutase are predicted to interfere with the structure and/or stability of the loop that carries His-627, the presumed lower axial ligand to the cobalt of adenosylcobalamin. Two mutants that lead to severe impairment (mut0) are Gly-630 --> Glu and Gly-703 --> Arg, which map to the binding site for the dimethylbenzimidazole nucleotide substituent of adenosylcobalamin. The substitution of larger residues for glycine is predicted to block the binding of adenosylcobalamin.
ISSN	00278424
e-ISSN	10916490
Journal	Proceedings of the National Academy of Sciences of the United States of America
Issue Number	11
Volume Number	93
Language	English
Publisher	National Academy of Sciences
Publisher Date	1996-07-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	5-Methyltetrahydrofolate-Homocysteine S-Methyltransferase Chemistry Methylmalonyl-CoA Mutase Genetics Point Mutation Protein Conformation Alleles Amino Acid Sequence Animals Bacteria Enzymology Binding Sites Caenorhabditis Elegans Escherichia Coli Heterozygote Metabolism, Inborn Errors Methylmalonic Acid Urine Metabolism Models, Molecular Molecular Sequence Data Nucleotides Polymorphism, Genetic Sequence Homology, Amino Acid Vitamin B 12 Comparative Study Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Multidisciplinary
Content Type	Text
Resource Type	Article
Subject	Multidisciplinary

Central Library (ISO-9001:2015 Certified)
Indian Institute of Technology Kharagpur
Kharagpur, West Bengal, India | PIN - 721302

See location in the Map
03222 282435
Mail: support@ndl.gov.in

Sl.	Authority	Responsibilities	Communication Details
1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
2	Indian Institute of Technology Kharagpur	Host Institute of the Project: The host institute of the project is responsible for providing infrastructure support and hosting the project	https://www.iitkgp.ac.in
3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
5	Website/Portal (Helpdesk)	Queries regarding NDLI and its services	support@ndl.gov.in
6	Contents and Copyright Issues	Queries related to content curation and copyright issues	content@ndl.gov.in
7	National Digital Library of India Club (NDLI Club)	Queries related to NDLI Club formation, support, user awareness program, seminar/symposium, collaboration, social media, promotion, and outreach	clubsupport@ndl.gov.in
8	Digital Preservation Centre (DPC)	Assistance with digitizing and archiving copyright-free printed books	dpc@ndl.gov.in
9	IDR Setup or Support	Queries related to establishment and support of Institutional Digital Repository (IDR) and IDR workshops	idr@ndl.gov.in