Compounds > cysteine

cysteine and Lysosomal Enzyme Disorders

cysteine has been researched along with Lysosomal Enzyme Disorders in 8 studies

Research

Studies (8)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's5 (62.50)18.2507
2000's1 (12.50)29.6817
2010's1 (12.50)24.3611
2020's1 (12.50)2.80

Authors

AuthorsStudies
Mashima, R; Nakanishi, M1
Courville, P; Quick, M; Reimer, RJ1
Sandhoff, K; Schepers, U; Schulze, H1
Ingendoh, A; Schmidt, B; Selmer, T; von Figura, K1
Ikonen, E; Jalanko, A; Lehto, VP; Peltonen, L; Riikonen, A; Sormunen, R1
Dierks, T; Schmidt, B; von Figura, K1
Itoh, K; Miharu, N; Mizoguchi, N; Ohama, K; Sakuraba, H; Shimmoto, M; Utsumi, K1
Enomaa, N; Ikonen, E; Peltonen, L; Ulmanen, I1

Reviews

1 review(s) available for cysteine and Lysosomal Enzyme Disorders

ArticleYear
Mammalian Sulfatases: Biochemistry, Disease Manifestation, and Therapy.
    International journal of molecular sciences, 2022, Jul-24, Volume: 23, Issue:15

    Topics: Animals; Cysteine; Female; Humans; Lysosomal Storage Diseases; Mammals; Pregnancy; Protein Processing, Post-Translational; Proteins; Sulfatases

2022

Other Studies

7 other study(ies) available for cysteine and Lysosomal Enzyme Disorders

ArticleYear
Structure-function studies of the SLC17 transporter sialin identify crucial residues and substrate-induced conformational changes.
    The Journal of biological chemistry, 2010, Jun-18, Volume: 285, Issue:25

    Topics: Amino Acid Sequence; Animals; Cysteine; Glucuronic Acid; Humans; Lysosomal Storage Diseases; Molecular Sequence Data; Mutation; N-Acetylneuraminic Acid; Organic Anion Transporters; Protein Isoforms; Protein Structure, Tertiary; Rats; Sequence Homology, Amino Acid; Structure-Activity Relationship; Symporters

2010
Overexpression and mass spectrometry analysis of mature human acid ceramidase.
    Biological chemistry, 2007, Volume: 388, Issue:12

    Topics: Animals; Baculoviridae; Blotting, Western; Cell Line; Culture Media; Cysteine; Disulfides; Electrophoresis, Polyacrylamide Gel; Galactosylgalactosylglucosylceramidase; Glycosylation; Humans; Hydrogen-Ion Concentration; Insecta; Kinetics; Lysosomal Storage Diseases; Mass Spectrometry; Oligosaccharides; Oxidation-Reduction; Peptides; Plasmids; Recombinant Proteins; Spectrometry, Mass, Electrospray Ionization; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Trypsin

2007
A novel amino acid modification in sulfatases that is defective in multiple sulfatase deficiency.
    Cell, 1995, Jul-28, Volume: 82, Issue:2

    Topics: Alanine; Aldehydes; Amino Acid Sequence; Animals; Cell Line; Conserved Sequence; Cysteine; DNA, Complementary; Fibroblasts; Humans; Lysosomal Storage Diseases; Molecular Sequence Data; Mutation; Sequence Homology, Amino Acid; Sulfatases

1995
Dissection of the molecular consequences of a double mutation causing a human lysosomal disease.
    DNA and cell biology, 1994, Volume: 13, Issue:3

    Topics: Amino Acid Sequence; Animals; Aspartylglucosaminuria; Aspartylglucosylaminase; Base Sequence; CHO Cells; Cricetinae; Cysteine; DNA, Complementary; Finland; Fluorescent Antibody Technique; Humans; Lysosomal Storage Diseases; Microscopy, Immunoelectron; Point Mutation; Transfection

1994
Conversion of cysteine to formylglycine: a protein modification in the endoplasmic reticulum.
    Proceedings of the National Academy of Sciences of the United States of America, 1997, Oct-28, Volume: 94, Issue:22

    Topics: Alanine; Amino Acid Sequence; Binding Sites; Biological Transport; Cerebroside-Sulfatase; Cysteine; Endoplasmic Reticulum; Glycine; Humans; Lysosomal Storage Diseases; Molecular Sequence Data; Protein Biosynthesis; Protein Processing, Post-Translational

1997
Protective protein/cathepsin A loss in cultured cells derived from an early-infantile form of galactosialidosis patients homozygous for the A1184-G transition (Y395C mutation).
    Biochemical and biophysical research communications, 1998, Jun-09, Volume: 247, Issue:1

    Topics: Amino Acid Substitution; Antibodies; Carboxypeptidases; Cathepsin A; Cells, Cultured; Cysteine; Female; Fluorescent Antibody Technique, Indirect; Genotype; Homozygote; Humans; Immunoblotting; Infant; Lysosomal Storage Diseases; Lysosomes; Male; Pedigree; Point Mutation; Tyrosine

1998
In vitro mutagenesis helps to unravel the biological consequences of aspartylglucosaminuria mutation.
    Genomics, 1991, Volume: 11, Issue:1

    Topics: Aspartylglucosylaminase; Base Sequence; Blotting, Western; Cell Line; Cloning, Molecular; Cysteine; Disulfides; DNA; DNA Mutational Analysis; Humans; Lysosomal Storage Diseases; Molecular Sequence Data; Mutagenesis

1991