PURIFICATION AND CHARACTERIZATION OF AN ACTIVATOR PROTEIN FOR THE DEGRADATION OF GLYCOLIPIDS G_M2 AND G_A2 BY HEXOSAMINIDASE ACONZELMANN E; SANDHOFF K.1979; Z. (HOPPE-SEYLER'S) PHYSIOL. CHEM.; DEU; DA. 1979; VOL. 360; NO 12; PP. 1837-1849; ABS. GER; BIBL. 27 REF.Article

AB VARIANT OF INFANTILE GM2 GANGLIOSIDOSIS: DEFICIENCY OF A FACTOR NECCESSARY FOR STIMULATION OF HEXOSAMINIDASE A-CATALYZED DEGRADATION OF GANGLIOSIDE GM2 AND GLYCOLIPID GA2CONZELMANN E; SANDHOFF K.1978; PROC. NATION. ACAD. SCI. USA; USA; DA. 1978; VOL. 75; NO 8; PP. 3979-3983; BIBL. 16 REF.Article

SPECIFICITY OF HUMAN LIVER HEXOSAMINIDASES A AND B AGAINST GLYCOSPHINGOLIPIDS G_M2 AND G_A2. PURIFICATION OF THE ENZYMES BY AFFINITY CHROMATOGRAPHY EMPLOYING SPECIFIC ELUTION.SANDHOFF K; CONZELMANN E; NEHRKORN H et al.1977; Z. (HOPPE-SEYLER'S) PHYSIOL. CHEM.; DTSCH.; DA. 1977; VOL. 358; NO 7; PP. 779-787; ABS. ALLEM.; BIBL. 32 REF.Article

Adult-onset metachromatic leucodystrophy presenting without psychiatric symptomsKLEMM, E; CONZELMANN, E.Journal of neurology. 1989, Vol 236, Num 7, pp 427-429, issn 0340-5354Article

PURIFICATION BIOCHEMICAL AND IMMUNOLOGICAL CHARACTERISATION OF HEXOSAMINIDASE A FROM VARIANT AB OF INFANTILE G_M2 GANGLIOSIDOSIS.CONZELMANN E; SANDHOFF K; NEHRKORN H et al.1978; EUROP. J. BIOCHEM.; GERM.; DA. 1978; VOL. 84; NO 1; PP. 27-33; BIBL. 27 REF.Article

LYSOSOMAL ENZYME PRECURSORS IN HUMAN FIBROBLASTS. ACTIVATION OF CATHEPSIN D PRECURSOR IN VITRO AND ACTIVITY OF BETA -HEXOSAMINIADASE A PRECURSOR TOWARDS GANGLIOSIDE G_M2HASILIK A; VON FIGURA K; CONZELMANN E et al.1982; EUR. J. BIOCHEM.; ISSN 0014-2956; DEU; DA. 1982; VOL. 125; NO 2; PP. 317-321; BIBL. 31 REF.Article

Glycolipid and glycoprotein degradationCONZELMANN, E; SANDHOFF, K.Advances in enzymology. 1987, Vol 60, pp 89-216, issn 0373-1871Article

Activator proteins for lysosomal glycolipid hydrolysisCONZELMANN, E; SANDHOFF, K.Methods of biochemical analysis. 1987, Vol 32, pp 1-23, issn 0076-6941Article

Assay for cerebroside sulfate (sulfatide) sulfatase in cultured skin fibroblasts with the natural activator proteinLEE-VAUPEL, M; CONZELMANN, E.Clinica chimica acta. 1987, Vol 168, Num 1, pp 55-68, issn 0009-8981Article

Biochemical basis of late-onset neurolipidosesCONZELMANN, E; SANDHOFF, K.Developmental neuroscience. 1991, Vol 13, Num 4-5, pp 197-204, issn 0378-5866Article

A simple chromogenic assay for arylsulfatase ALEE-VAUPEL, M; CONZELMANN, E.Clinica chimica acta. 1987, Vol 164, Num 2, pp 171-180, issn 0009-8981Article

Partial enzyme deficiencies: residual activities and the development of neurological disordersCONZELMANN, E; SANDHOFF, K.Developmental neuroscience. 1983, Vol 6, Num 1, pp 58-71, issn 0378-5866Article

COMPLEXING OF GLYCOLIPIDS AND THEIR TRANSFER BETWEEN MEMBRANES BY THE ACTIVATOR PROTEIN FOR DEGRADATION OF LYSOSOMAL GANGLIOSIDE GM₂CONZELMANN E; BURG J; STEPHAN G et al.1982; EUR. J. BIOCHEM.; ISSN 0014-2956; DEU; DA. 1982; VOL. 123; NO 2; PP. 455-464; BIBL. 35 REF.Article

Quantitative correlation between the residual activity of β-hexosaminidase A and arysulfatase A and the severity of the resulting lysosomal storage diseaseLEINEKUGEL, P; MICHEL, S; CONZELMANN, E et al.Human genetics. 1992, Vol 88, Num 5, pp 513-523, issn 0340-6717Article

Activator protein for the degradation of globotriaosylceramide by human α-galactosidaseGÄRTNER, S; CONZELMANN, E; SANDHOFF, K et al.The Journal of biological chemistry (Print). 1983, Vol 258, Num 20, pp 12378-12385, issn 0021-9258Article

Accumulation of phytanic acid α-oxidation intermediates in Zellweger fibroblastsFINGERHUT, R; SCHMITZ, W; CONZELMANN, E et al.Journal of inherited metabolic disease. 1993, Vol 16, Num 3, pp 591-594, issn 0141-8955Article

Adrenoleukodystrophie bei einem Geschwiserpaar = Leucodystrophie surrénalienne chez 2 frères = Adrenoleukodystrophy in two siblingsROSSKAMP, R; FICHSEL, H; RÖDER, B et al.Klinische Pädiatrie. 1985, Vol 197, Num 1, pp 61-64, issn 0300-8630Article

Hereditary heat-labile hexosaminidase B: a variant whose homozygotes synthesize a functional HEX ANAVON, R; KOPEL, R; NUTMAN, J et al.American journal of human genetics. 1985, Vol 37, Num 1, pp 138-146, issn 0002-9297Article

Degradation of gangliosides by the lysosomal sialidase requires an activator proteinFINGERHUT, R; VAN DER HORST, G. T. J; VERHEIJEN, F. W et al.European journal of biochemistry (Print). 1992, Vol 208, Num 3, pp 623-629, issn 0014-2956Article

Pseudodeficiency of arylsulfatase A: a common genetic polymorphism with possible disease implicationsHOHENSCHUTZ, C; EICH, P; FRIEDL, W et al.Human genetics. 1989, Vol 82, Num 1, pp 45-48, issn 0340-6717, 4 p.Article

Incorporation and metabolism of ganglioside G_M2 in skin fibroblasts from normal and G_M2 gangliosidosis subjectsSONDERFELD, S; CONZELMANN, E; SCHWARZMANN, G et al.European journal of biochemistry (Print). 1985, Vol 149, Num 2, pp 247-255, issn 0014-2956Article

Mapping of the gene coding for the human G_M2 activator protein to chromosome 5BURG, J; CONZELMANN, E; SANDHOFF, K et al.Annals of human genetics. 1985, Vol 49, Num 1, pp 41-45, issn 0003-4800Article

Prenatal diagnosis of X-linked adrenoleukodystrophy combining biochemical, immunocytochemical and DNA analysesMAIER, E. M; ROSCHER, A. A; KAMMERER, S et al.Prenatal diagnosis. 1999, Vol 19, Num 4, pp 364-368, issn 0197-3851Article

Genotype-phenotype relationship in various degrees of arylsulfatase A deficiencyKAPPLER, J; LEINEKUGEL, P; CONZELMANN, E et al.Human genetics. 1991, Vol 86, Num 5, pp 463-470, issn 0340-6717Article

Low arylsumphatase A activity and choreoathetotic syndrome in three siblings : differentiation of pseudodeficiency from metachromatic leukodystrophyKAPPLER, J; WATTS, R. W. E; CONZELMANN, E et al.European journal of pediatrics. 1991, Vol 150, Num 4, pp 287-290, issn 0340-6199Article