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E. Conzelmann · SciRah

Researcher profile · Semantic Scholar

E. Conzelmann

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E. Conzelmann — researcher profile on SciRah

h-index: 26
Works: 64
Citations: 3,748

Most-cited papers

Mutations in VKORC1 cause warfarin resistance and multiple coagulation factor deficiency type 2
S. Rost, A. Fregin, V. Ivaškevičius, E. Conzelmann +9 more
2004Nature1,187 citations
Semantic Scholar
Partial enzyme deficiencies: residual activities and the development of neurological disorders.
E. Conzelmann, K. Sandhoff
1983Developmental Neuroscience258 citations
Semantic Scholar
AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2.
E. Conzelmann, Konrad Sandhoff
1978Proceedings of the National Academy of Sciences of the United States of America235 citations Open access
Semantic Scholar
Quantitative correlation between the residual activity of β-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease
P. Leinekugel, S. Michel, E. Conzelmann, K. Sandhoff
1992Human Genetics179 citations
Semantic Scholar
Incorporation and metabolism of ganglioside GM2 in skin fibroblasts from normal and GM2 gangliosidosis subjects.
S. Sonderfeld, E. Conzelmann, Gunter Schwarzmann, J. Burg +2 more
1985European Journal of Biochemistry142 citations
Semantic Scholar
Purification and characterization of an alpha-methylacyl-CoA racemase from human liver.
W. Schmitz, Christine Albers, R. Fingerhut, E. Conzelmann
1995European Journal of Biochemistry137 citations
Semantic Scholar
Purification and properties of an alpha-methylacyl-CoA racemase from rat liver.
Werner Schmitz, Ralph Fingerhut, E. Conzelmann
1994European Journal of Biochemistry119 citations
Semantic Scholar
Leukodystrophy incidence in Germany.
P. Heim, M. Claussen, Béatrice Hoffmann, E. Conzelmann +6 more
1997American journal of medical genetics118 citations
Semantic Scholar
Peroxisomal multifunctional enzyme of beta-oxidation metabolizing D-3-hydroxyacyl-CoA esters in rat liver: molecular cloning, expression and characterization.
Yong-Mei Qin, M. Poutanen, Heli M. Helander, Ari-Pekka Kvist +5 more
1997Biochemical Journal118 citations Open access
Semantic Scholar
A mouse model for alpha-methylacyl-CoA racemase deficiency: adjustment of bile acid synthesis and intolerance to dietary methyl-branched lipids.
K. Savolainen, Tiina J. Kotti, W. Schmitz, T. Savolainen +5 more
2004Human Molecular Genetics79 citations
Semantic Scholar
PEX1 Mutations in Complementation Group 1 of Zellweger Spectrum Patients Correlate with Severity of Disease
N. Preuß, U. Brosius, Martina Biermanns, A. Muntau +2 more
2002Pediatric Research71 citations
Semantic Scholar
Elevated α-Methylacyl-CoA Racemase Enzymatic Activity in Prostate Cancer
Chandan Kumar-Sinha, R. Shah, Bharathi Laxman, S. Tomlins +7 more
200470 citations Open access
Semantic Scholar
Ganglioside GM2 N-acetyl-beta-D-galactosaminidase activity in cultured fibroblasts of late-infantile and adult GM2 gangliosidosis patients and of healthy probands with low hexosaminidase level.
E. Conzelmann, H. Kytzia, R. Navon, Konrad Sandhoff
1983American Journal of Human Genetics69 citations
Semantic Scholar
In Mouse α-Methylacyl-CoA Racemase, the Same Gene Product Is Simultaneously Located in Mitochondria and Peroxisomes*
Tiina J. Kotti, K. Savolainen, Heli M. Helander, A. Yagi +5 more
2000Journal of Biological Chemistry68 citations Open access
Semantic Scholar
Mitochondrial 2,4-dienoyl-CoA Reductase Deficiency in Mice Results in Severe Hypoglycemia with Stress Intolerance and Unimpaired Ketogenesis
I. Miinalainen, W. Schmitz, A. Huotari, Kaija J. Autio +6 more
2009PLoS Genetics66 citations Open access
Semantic Scholar
The human IgM antibody SAM-6 induces tumor-specific apoptosis with oxidized low-density lipoprotein
S. Brändlein, Nicole Rauschert, L. Rasche, A. Dreykluft +4 more
2007Molecular Cancer Therapeutics60 citations Open access
Semantic Scholar
Proteomic analysis of cathepsin B and L-deficient mouse brain lysosomes
Sonja Stahl, Yvonne Reinders, E. Asan, W. Mothes +3 more
2007Biochimica et Biophysica Acta58 citations Open access
Semantic Scholar
The Biochemical Basis of Gangliosidoses
K. Sandhoff, E. Conzelmann
1984Neuropediatrics57 citations
Semantic Scholar
Compound heterozygosity for metachromatic leukodystrophy and arylsulfatase A pseudodeficiency alleles is not associated with progressive neurological disease.
J. Penzien, J. Kappler, N. Herschkowitz, B. Schuknecht +8 more
1993American Journal of Human Genetics56 citations
Semantic Scholar
Biochemical basis of late-onset neurolipidoses.
E. Conzelmann, K. Sandhoff
1991Developmental Neuroscience49 citations
Semantic Scholar
Activator protein for the degradation of globotriaosylceramide by human alpha-galactosidase.
S. Gärtner, E. Conzelmann, K. Sandhoff
1983Journal of Biological Chemistry42 citations Open access
Semantic Scholar
α-Methylacyl-CoA Racemase from Mycobacterium tuberculosis
K. Savolainen, P. Bhaumik, W. Schmitz, Tiina J. Kotti +3 more
2005Journal of Biological Chemistry41 citations Open access
Semantic Scholar
The catalysis of the 1,1-proton transfer by alpha-methyl-acyl-CoA racemase is coupled to a movement of the fatty acyl moiety over a hydrophobic, methionine-rich surface.
P. Bhaumik, W. Schmitz, A. Hassinen, J. Hiltunen +2 more
2007Journal of Molecular Biology40 citations
Semantic Scholar
The role of α-methylacyl-CoA racemase in bile acid synthesis
Dean A. Cuebas, Christopher Phillips, W. Schmitz, E. Conzelmann +1 more
200238 citations Open access
Semantic Scholar
Elevated alpha-methylacyl-CoA racemase enzymatic activity in prostate cancer.
Chandan Kumar-Sinha, R. Shah, Bharathi Laxman, S. Tomlins +7 more
2004American Journal of Pathology37 citations
Semantic Scholar
Defects in degradation of blood group A and B glycosphingolipids in Schindler and Fabry diseases the nomenclature used for neutral glycolipids follows the IUPAC-IUB recommendation (1). DOI 10.1194/jlr.M100423-JLR200
B. Asfaw, J. Ledvinová, R. Dobrovolný, H. D. Bakker +8 more
2002Journal of Lipid Research33 citations Open access
Semantic Scholar
Activation of lysosomal hydrolysis of complex glycolipids by non-enzymic proteins
K. Sandhoff, E. Conzelmann
197926 citations
Semantic Scholar
Peroxisomal membrane channel Pxmp2 in the mammary fat pad is essential for stromal lipid homeostasis and for development of mammary gland epithelium in mice.
Miia H. Vapola, A. Rokka, Raija T. Sormunen, L. Alhonen +6 more
2014Developmental Biology25 citations Open access
Semantic Scholar
Genotype-phenotype relationship in various degrees of arylsulfatase A deficiency
J. Kappler, P. Leinekugel, E. Conzelmann, W. Kleijer +5 more
1991Human Genetics24 citations
Semantic Scholar
Pseudodeficiency of arylsulfatase A: a common genetic polymorphism with possible disease implications
C. Hohenschutz, P. Eich, W. Friedl, A. Waheed +2 more
1989Human Genetics23 citations
Semantic Scholar
Crystallization and preliminary X-ray diffraction studies of an alpha-methylacyl-CoA racemase from Mycobacterium tuberculosis.
P. Bhaumik, P. Kursula, Ville Ratas, E. Conzelmann +3 more
2003Acta Crystallographica Section D: Biological Crystallography19 citations
Semantic Scholar
The role of alpha-methylacyl-CoA racemase in bile acid synthesis.
Dean A. Cuebas, Christopher Phillips, W. Schmitz, E. Conzelmann +1 more
2002Biochemical Journal18 citations
Semantic Scholar
The enolization chemistry of a thioester-dependent racemase: the 1.4 Å crystal structure of a reaction intermediate complex characterized by detailed QM/MM calculations.
Satyan Sharma, P. Bhaumik, W. Schmitz, R. Venkatesan +4 more
2012Journal of Physical Chemistry B17 citations
Semantic Scholar
Prenatal diagnosis of X‐linked adrenoleukodystrophy combining biochemical, immunocytochemical and DNA analyses
E. Maier, A. Roscher, Stefan Kammerer, K. Mehnert +2 more
1999Prenatal Diagnosis16 citations
Semantic Scholar
Low arylsulphatase A activity and choreoathetotic syndrome in three siblings: differentiation of pseudodeficiency from metachromatic leukodystrophy
J. Kappler, R. W. Watts, E. Conzelmann, D. Gibbs +2 more
1991European Journal of Pediatrics16 citations
Semantic Scholar
Assay for cerebroside sulfate (sulfatide) sulfatase in cultured skin fibroblasts with the natural activator protein.
M. Lee-Vaupel, E. Conzelmann
1987Clinica chimica acta; international journal of clinical chemistry13 citations
Semantic Scholar
Fundamentals of Ganglioside Catabolism
K. Sandhoff, G. Schwarzmann, F. Sarmientos, E. Conzelmann
198713 citations
Semantic Scholar
Prenatal Diagnosis of GM2 Gangliosidosis with High Residual Hexosaminidase A Activity (Variant B1; Pseudo AB Variant)
E. Conzelmann, H. Nehrkorn, H. Kytzia, Konrad Sandhoff +5 more
1985Pediatric Research12 citations
Semantic Scholar
Molecular cloning of cDNA species for rat and mouse liver α-methylacyl-CoA racemases
W. Schmitz, H. Helander, J. Hiltunen, E. Conzelmann
199711 citations
Semantic Scholar
Hereditary heat-labile hexosaminidase B: a variant whose homozygotes synthesize a functional HEX A.
Ruth Navon, R. Kopel, J. Nutman, A. Frisch +3 more
1985American Journal of Human Genetics11 citations
Semantic Scholar
Insertion into cultured cells and metabolism and intracellular transport of exogenous gangliosides.
G. Schwarzmann, S. Sonderfeld, E. Conzelmann, D. Marsh +1 more
198411 citations
Semantic Scholar
Metabolic adaptation allows Amacr-deficient mice to remain symptom-free despite low levels of mature bile acids.
Eija M. Selkälä, Sanna M. Kuusisto, T. Salonurmi, M. Savolainen +9 more
2013Biochimica et Biophysica Acta10 citations
Semantic Scholar
Adult-onset metachromatic leucodystrophy presenting without psychiatric symptoms
E. Klemm, E. Conzelmann
1989Journal of Neurology10 citations
Semantic Scholar
The Physiological Roles of Activator Proteins for Lysosomal Glycolipid Degradation
E. Conzelmann, M. Lee-Vaupel, K. Sandhoff
19889 citations
Semantic Scholar
The specificity of human N-acetyl-beta-D-hexosaminidases towards glycosphincolipids is determined by an activator protein.
E. Conzelmann, Konrad Sandhoff
1980Advances in Experimental Medicine and Biology8 citations
Semantic Scholar
AB variant of infantile GM 2 gangliosidosis : Deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM 2 and glycolipid GA 2 ( lipid storage disease / activator deficiency )
E. Conzelmann, And, K. Sandhoff
5 citations
Semantic Scholar
Glycosphingolipid Activator Proteins
W. Fürst, A. Vogel, M. Lee-Vaupel, E. Conzelmann +1 more
19864 citations
Semantic Scholar
The Role of Activator Proteins in Glycolipid Degradation and Storage Diseases
E. Conzelmann
19893 citations
Semantic Scholar
CONCEPTS OF GANGLIOSIDE METABOLISM
U. Hinrichs, S. Sonderfeld, G. Schwarzmann, E. Conzelmann +1 more
19863 citations
Semantic Scholar
Biochemistry of Gangliosidoses
K. Sandhoff, E. Conzelmann
19853 citations
Semantic Scholar

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