Lista de obras - Kurt von Figura - Dominio Público Uruguay

A general binding mechanism for all human sulfatases by the formylglycine-generating enzyme

artículo científico publicado en 2005

A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesis

artículo científico publicado en 2003

Crystal structure of human pFGE, the paralog of the Calpha-formylglycine-generating enzyme

artículo científico publicado en 2005

Defective oligomerization of arylsulfatase a as a cause of its instability in lysosomes and metachromatic leukodystrophy

artículo científico publicado en 2002

Deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type Ik.

artículo científico publicado en 2004

Deficiency of UDP-galactose:N-acetylglucosamine beta-1,4-galactosyltransferase I causes the congenital disorder of glycosylation type IId.

artículo científico publicado en 2002

Deficiency of dolichyl-P-Man:Man7GlcNAc2-PP-dolichyl mannosyltransferase causes congenital disorder of glycosylation type Ig

artículo científico publicado en 2002

Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts

artículo científico publicado en 2004

Efficacy of enzyme replacement therapy in alpha-mannosidosis mice: a preclinical animal study

artículo científico publicado en 2004

Expression, localization, structural, and functional characterization of pFGE, the paralog of the Calpha-formylglycine-generating enzyme

artículo científico publicado en 2005

Granzyme-mediated cytotoxicity does not involve the mannose 6-phosphate receptors on target cells

artículo científico publicado en 2004

Heparan sulfate 6-O-endosulfatases: discrete in vivo activities and functional co-operativity

artículo científico publicado en 2006

Identification of formylglycine in sulfatases by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry

artículo científico publicado en 2003

Identification of novel lysosomal matrix proteins by proteome analysis

artículo científico publicado en 2005

Involvement of cathepsin E in exogenous antigen processing in primary cultured murine microglia

scientific journal article

LIMP-2/LGP85 deficiency causes ureteric pelvic junction obstruction, deafness and peripheral neuropathy in mice

artículo científico publicado en 2003

Locating the anomalous scatterer substructures in halide and sulfur phasing

artículo científico publicado en 2003

Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation

artículo científico publicado en 2005

Molecular analysis of SUMF1 mutations: stability and residual activity of mutant formylglycine-generating enzyme determine disease severity in multiple sulfatase deficiency

artículo científico publicado en 2008

Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme

artículo científico publicado en 2005

Molecular basis of multiple sulfatase deficiency, mucolipidosis II/III and Niemann-Pick C1 disease - Lysosomal storage disorders caused by defects of non-lysosomal proteins

artículo científico publicado en 2008

Molecular characterization of the human Calpha-formylglycine-generating enzyme

artículo científico publicado en 2005

Molecular characterization of the hypothetical 66.3-kDa protein in mouse: lysosomal targeting, glycosylation, processing and tissue distribution

scientific journal article

Multiple sulfatase deficiency is caused by mutations in the gene encoding the human C(alpha)-formylglycine generating enzyme

scientific journal article

Paralog of the formylglycine-generating enzyme--retention in the endoplasmic reticulum by canonical and noncanonical signals

artículo científico publicado en 2008

Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease).

artículo científico publicado en 2005

Phosphorylation of the AP2 mu subunit by AAK1 mediates high affinity binding to membrane protein sorting signals

artículo científico publicado en 2002

Structural requirements for interactions between leucine-sorting signals and clathrin-associated adaptor protein complex AP3

artículo científico publicado en 2002

Targeted disruption of the mouse phosphomannomutase 2 gene causes early embryonic lethality

artículo científico publicado en 2006

The disintegrin/metalloprotease ADAM 10 is essential for Notch signalling but not for alpha-secretase activity in fibroblasts

artículo científico publicado en 2002

The human SUMF1 gene, required for posttranslational sulfatase modification, defines a new gene family which is conserved from pro- to eukaryotes ⬇️

artículo científico publicado el 16 de octubre de 2003

The non-catalytic N-terminal extension of formylglycine-generating enzyme is required for its biological activity and retention in the endoplasmic reticulum

artículo científico publicado en 2008

The tyrosine motifs of Lamp 1 and LAP determine their direct and indirect targetting to lysosomes

artículo científico publicado en 2002

Thyroid functions of mouse cathepsins B, K, and L.

artículo científico publicado en 2003

Lista de obras de Kurt von Figura

A general binding mechanism for all human sulfatases by the formylglycine-generating enzyme

A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesis

Crystal structure of human pFGE, the paralog of the Calpha-formylglycine-generating enzyme

Defective oligomerization of arylsulfatase a as a cause of its instability in lysosomes and metachromatic leukodystrophy

Deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type Ik.

Deficiency of UDP-galactose:N-acetylglucosamine beta-1,4-galactosyltransferase I causes the congenital disorder of glycosylation type IId.

Deficiency of dolichyl-P-Man:Man7GlcNAc2-PP-dolichyl mannosyltransferase causes congenital disorder of glycosylation type Ig

Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts

Efficacy of enzyme replacement therapy in alpha-mannosidosis mice: a preclinical animal study

Expression, localization, structural, and functional characterization of pFGE, the paralog of the Calpha-formylglycine-generating enzyme

Granzyme-mediated cytotoxicity does not involve the mannose 6-phosphate receptors on target cells

Heparan sulfate 6-O-endosulfatases: discrete in vivo activities and functional co-operativity

Identification of formylglycine in sulfatases by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry

Identification of novel lysosomal matrix proteins by proteome analysis

Involvement of cathepsin E in exogenous antigen processing in primary cultured murine microglia

LIMP-2/LGP85 deficiency causes ureteric pelvic junction obstruction, deafness and peripheral neuropathy in mice

Locating the anomalous scatterer substructures in halide and sulfur phasing

Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation

Molecular analysis of SUMF1 mutations: stability and residual activity of mutant formylglycine-generating enzyme determine disease severity in multiple sulfatase deficiency

Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme

Molecular basis of multiple sulfatase deficiency, mucolipidosis II/III and Niemann-Pick C1 disease - Lysosomal storage disorders caused by defects of non-lysosomal proteins

Molecular characterization of the human Calpha-formylglycine-generating enzyme

Molecular characterization of the hypothetical 66.3-kDa protein in mouse: lysosomal targeting, glycosylation, processing and tissue distribution

Multiple sulfatase deficiency is caused by mutations in the gene encoding the human C(alpha)-formylglycine generating enzyme

Paralog of the formylglycine-generating enzyme--retention in the endoplasmic reticulum by canonical and noncanonical signals

Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease).

Phosphorylation of the AP2 mu subunit by AAK1 mediates high affinity binding to membrane protein sorting signals

Structural requirements for interactions between leucine-sorting signals and clathrin-associated adaptor protein complex AP3

Targeted disruption of the mouse phosphomannomutase 2 gene causes early embryonic lethality

The disintegrin/metalloprotease ADAM 10 is essential for Notch signalling but not for alpha-secretase activity in fibroblasts

The human SUMF1 gene, required for posttranslational sulfatase modification, defines a new gene family which is conserved from pro- to eukaryotes ⬇️

The non-catalytic N-terminal extension of formylglycine-generating enzyme is required for its biological activity and retention in the endoplasmic reticulum

The tyrosine motifs of Lamp 1 and LAP determine their direct and indirect targetting to lysosomes

Thyroid functions of mouse cathepsins B, K, and L.