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Hugo Moser

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Steven J Steinberg; A Snowden; Nancy Braverman; L Chen; Paul A Watkins; P T Clayton; K D R Setchell; James Heubi; Gerald V Raymond; Ann B Moser; Hugo W Moser
A PEX10 defect in a patient with no detectable defect in peroxisome assembly or metabolism in cultured fibroblasts.
Joseph P Pillion; Hugo W Moser; Gerald V Raymond
Auditory function in adrenomyeloneuropathy.
Hugo W Moser; Ann B Moser; Kim Hollandsworth; N Hong Brereton; Gerald V Raymond
"Lorenzo's oil" therapy for X-linked adrenoleukodystrophy: rationale and current assessment of efficacy.

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2009: Steven J Steinberg; A Snowden; Nancy Braverman; L Chen; Paul A Watkins; P T Clayton; K D R Setchell; James Heubi; Gerald V Raymond; Ann B Moser; Hugo W Moser
A PEX10 defect in a patient with no detectable defect in peroxisome assembly or metabolism in cultured fibroblasts.
Journal of inherited metabolic disease 2009;32(1):109-19.
2008: Joseph P Pillion; Hugo W Moser; Gerald V Raymond
Auditory function in adrenomyeloneuropathy.
Journal of the neurological sciences 2008;269(1-2):24-9.
2007: Hugo W Moser; Ann B Moser; Kim Hollandsworth; N Hong Brereton; Gerald V Raymond
"Lorenzo's oil" therapy for X-linked adrenoleukodystrophy: rationale and current assessment of efficacy.
Journal of molecular neuroscience : MN 2007;33(1):105-13.
2007: Asif Mahmood; Gerald V Raymond; Prachi Dubey; Charles Peters; Hugo W Moser
Survival analysis of haematopoietic cell transplantation for childhood cerebral X-linked adrenoleukodystrophy: a comparison study.
Lancet neurology 2007;6(8):687-92.
2007: Florian Eichler; Asif Mahmood; Daniel J Loes; Lena Bezman; Doris D M Lin; Hugo W Moser; Gerald V Raymond
Magnetic resonance imaging detection of lesion progression in adult patients with X-linked adrenoleukodystrophy.
Archives of neurology 2007;64(5):659-64.
2007: Hugo W Moser; Asif Mahmood; Gerald V Raymond
X-linked adrenoleukodystrophy.
Nature clinical practice. Neurology 2007;3(3):140-51.
2006: Steven J Steinberg; Gabriele Dodt; Gerald V Raymond; Nancy Braverman; Ann B Moser; Hugo W Moser
Peroxisome biogenesis disorders.
Biochimica et biophysica acta 2006;1763(12):1733-48.
2006: Kathleen M Zackowski; Prachi Dubey; Gerald V Raymond; Susumu Mori; Amy J Bastian; Hugo W Moser
Sensorimotor function and axonal integrity in adrenomyeloneuropathy.
Archives of neurology 2006;63(1):74-80.
2006: Christiane Sylvia Cox; Prachi Dubey; Gerald V Raymond; Asif Mahmood; Ann B Moser; Hugo W Moser
Cognitive evaluation of neurologically asymptomatic boys with X-linked adrenoleukodystrophy.
Archives of neurology 2006;63(1):69-73.
2005: Hugo W Moser; Gerald V Raymond; Prachi Dubey
Adrenoleukodystrophy: new approaches to a neurodegenerative disease.
JAMA : the journal of the American Medical Association 2005;294(24):3131-4.
2005: Prachi Dubey; S. Ali Fatemi; Hao Huang; Lidia M Nagae-Poetscher; Setsu Wakana; Peter B Barker; Peter C M van Zijl; Hugo W Moser; Susumu Mori; Gerald V Raymond
Diffusion tensor-based imaging reveals occult abnormalities in adrenomyeloneuropathy.
Annals of neurology 2005;58(5):758-66.
2005: Hugo W Moser; Gerald V Raymond; Shou-En Lu; Larry R Muenz; Ann B Moser; Jiahong Xu; Richard O Jones; Daniel J Loes; Elias Melhem; Prachi Dubey; Lena Bezman; N Hong Brereton; Augusto Odone
Follow-up of 89 asymptomatic patients with adrenoleukodystrophy treated with Lorenzo's oil.
Archives of neurology 2005;62(7):1073-80.
2005: Seth A Smith; Xavier Golay; S. Ali Fatemi; Craig K Jones; Gerald V Raymond; Hugo W Moser; Peter C M van Zijl
Magnetization transfer weighted imaging in the upper cervical spinal cord using cerebrospinal fluid as intersubject normalization reference (MTCSF imaging).
Magnetic resonance in medicine : official journal of the Society of Magnetic Resonance in Medicine / Society of Magnetic Resonance in Medicine 2005;54(1):201-6.
2005: S. Ali Fatemi; Seth A Smith; Prachi Dubey; Kathleen M Zackowski; Amy J Bastian; Peter C M van Zijl; Hugo W Moser; Gerald V Raymond; Xavier Golay
Magnetization transfer MRI demonstrates spinal cord abnormalities in adrenomyeloneuropathy.
Neurology 2005;64(10):1739-45.
2005: Prachi Dubey; Gerald V Raymond; Ann B Moser; Sidharth Kharkar; Lena Bezman; Hugo W Moser
Adrenal insufficiency in asymptomatic adrenoleukodystrophy patients identified by very long-chain fatty acid screening.
The Journal of pediatrics 2005;146(4):528-32.
2005: Prachi Dubey; S. Ali Fatemi; Peter B Barker; Mahaveer Degaonkar; M Troeger; Kathleen M Zackowski; Amy J Bastian; Seth A Smith; Martin G Pomper; Hugo W Moser; Gerald V Raymond
Spectroscopic evidence of cerebral axonopathy in patients with "pure" adrenomyeloneuropathy.
Neurology 2005;64(2):304-10.
2004: Hugo W Moser
Genetic causes of mental retardation.
Annals of the New York Academy of Sciences 2004;1038():44-8.
2004: Charles Peters; Lawrence Charnas; Ye Tan; Richard S Ziegler; Elsa G Shapiro; Todd E DeFor; Satkiran S Grewal; Paul J Orchard; Susan L Abel; Anne I Goldman; Norma K C Ramsay; Kathryn E Dusenbery; Daniel J Loes; Lawrence A Lockman; Shunichi Kato; Patrick R Aubourg; Hugo W Moser; William Krivit
Cerebral X-linked adrenoleukodystrophy: the international hematopoietic cell transplantation experience from 1982 to 1999.
Blood 2004;104(3):881-8.
2004: Hugo W Moser; S. Ali Fatemi; Kathleen M Zackowski; Seth A Smith; Xavier Golay; Larry R Muenz; Gerald V Raymond
Evaluation of therapy of X-linked adrenoleukodystrophy.
Neurochemical research 2004;29(5):1003-16.
2003: Francesca Pizzini; S. Ali Fatemi; Peter B Barker; Lidia M Nagae-Poetscher; ALENA HORSKA; Andrew W Zimmerman; Hugo W Moser; Genila Bibat; Sakkubai Naidu
Proton MR spectroscopic imaging in Pelizaeus-Merzbacher disease.
AJNR. American journal of neuroradiology 2003;24(8):1683-9.
2003: Daniel J Loes; S. Ali Fatemi; Elias Melhem; N Gupte; Lena Bezman; Hugo W Moser; Gerald V Raymond
Analysis of MRI patterns aids prediction of progression in X-linked adrenoleukodystrophy.
Neurology 2003;61(3):369-74.
2003: S. Ali Fatemi; Peter B Barker; Aziz Ulug; Lidia M Nagae-Poetscher; N J Beauchamp; Ann B Moser; Gerald V Raymond; Hugo W Moser; Sakkubai Naidu
MRI and proton MRSI in women heterozygous for X-linked adrenoleukodystrophy.
Neurology 2003;60(8):1301-7.
2003: Hugo W Moser; Gerald V Raymond; Wolfgang Koehler; Piotr Sokolowski; Folker Hanefeld; Georg Christoph Korenke; Anne Green; Daniel J Loes; Donald H Hunneman; Richard O Jones; Shou-En Lu; Graziella Uziel; Marisa L Giros; Frank Roels
Evaluation of the preventive effect of glyceryl trioleate-trierucate ("Lorenzo's oil") therapy in X-linked adrenoleukodystrophy: results of two concurrent trials.
Advances in experimental medicine and biology 2003;544():369-87.
2002: Florian Eichler; Ryuta Itoh; Peter B Barker; Susumu Mori; Elizabeth Garrett-Mayer; Peter C M van Zijl; Hugo W Moser; Gerald V Raymond; Elias Melhem
Proton MR spectroscopic and diffusion tensor brain MR imaging in X-linked adrenoleukodystrophy: initial experience.
Radiology 2002;225(1):245-52.
2002: Yumi Tagawa; Wouter Laroy; Leonardo Nimrichter; Susan E Fromholt; Ann B Moser; Hugo W Moser; Ronald Schnaar
Anti-ganglioside antibodies bind with enhanced affinity to gangliosides containing very long chain fatty acids.
Neurochemical research 2002;27(7-8):847-55.
2002: Patricia M Villemagne; Sakkubai Naidu; Victor L Villemagne; Myron Yaster; Henry N Wagner; James C Harris; Hugo W Moser; Michael V Johnston; Robert F Dannals; Dean F Wong
Brain glucose metabolism in Rett Syndrome.
Pediatric neurology 2002;27(2):117-22.
2002: Deyanira Corzo; William Gibson; Kisha Johnson; Grant Mitchell; Guy LePage; Gerald F Cox; Robin Casey; Carolyn Zeiss; Heidi Tyson; Garry R Cutting; Gerald V Raymond; Kirby D Smith; Paul A Watkins; Ann B Moser; Hugo W Moser; Steven J Steinberg
Contiguous deletion of the X-linked adrenoleukodystrophy gene (ABCD1) and DXS1357E: a novel neonatal phenotype similar to peroxisomal biogenesis disorders.
American journal of human genetics 2002;70(6):1520-31.
2002: Florian Eichler; Peter B Barker; Christiane Sylvia Cox; David Edwin; Aziz Ulug; Hugo W Moser; Gerald V Raymond
Proton MR spectroscopic imaging predicts lesion progression on MRI in X-linked adrenoleukodystrophy.
Neurology 2002;58(6):901-7.
2002: Susumu Mori; Walter E Kaufmann; Christos Davatzikos; Bram Stieltjes; Laura Amodei; Kim Fredericksen; Godfrey D Pearlson; Elias Melhem; Meiyappan Solaiyappan; Gerald V Raymond; Hugo W Moser; Peter C M van Zijl
Imaging cortical association tracts in the human brain using diffusion-tensor-based axonal tracking.
Magnetic resonance in medicine : official journal of the Society of Magnetic Resonance in Medicine / Society of Magnetic Resonance in Medicine 2002;47(2):215-23.
2001: Stephan Kemp; A Pujol; Hans R Waterham; Bjorn M Van Geel; Corinne D Boehm; Gerald V Raymond; Garry R Cutting; Ronald J A Wanders; Hugo W Moser
ABCD1 mutations and the X-linked adrenoleukodystrophy mutation database: role in diagnosis and clinical correlations.
Human mutation 2001;18(6):499-515.
2001: Sakkubai Naidu; Walter E Kaufmann; Michael T Abrams; Godfrey D Pearlson; Diane C Lanham; Kim Fredericksen; Peter B Barker; ALENA HORSKA; Xavier Golay; Susumu Mori; Dean F Wong; M Yablonski; Hugo W Moser; Michael V Johnston
Neuroimaging studies in Rett syndrome.
Brain & development 2001;23 Suppl 1():S62-71.
2001: H M Su; Ann B Moser; Hugo W Moser; Paul A Watkins
Peroxisomal straight-chain Acyl-CoA oxidase and D-bifunctional protein are essential for the retroconversion step in docosahexaenoic acid synthesis.
The Journal of biological chemistry 2001;276(41):38115-20.
2001: Paul A Watkins; James A Hamilton; A Leaf; A A Spector; S A Moore; R E Anderson; Hugo W Moser; M J Noetzel; R Katz
Brain uptake and utilization of fatty acids: applications to peroxisomal biogenesis diseases.
Journal of molecular neuroscience : MN 2001;16(2-3):87-92; discussion 151-7.
2001: Lena Bezman; Ann B Moser; Gerald V Raymond; P Rinaldo; Paul A Watkins; Kirby D Smith; Nancy Kass; Hugo W Moser
Adrenoleukodystrophy: incidence, new mutation rate, and results of extended family screening.
Annals of neurology 2001;49(4):512-7.
2001: Elias Melhem; Thaddaeus Gotwald; R Itoh; S James Zinreich; Hugo W Moser
T2 relaxation measurements in X-linked adrenoleukodystrophy performed using dual-echo fast fluid-attenuated inversion recovery MR imaging.
AJNR. American journal of neuroradiology 2001;22(4):773-6.
2001: Ryuta Ito; Elias Melhem; Susumu Mori; Florian Eichler; Gerald V Raymond; Hugo W Moser
Diffusion tensor brain MR imaging in X-linked cerebral adrenoleukodystrophy.
Neurology 2001;56(4):544-7.
2001: Bjorn M Van Geel; Lena Bezman; Daniel J Loes; Hugo W Moser; Gerald V Raymond
Evolution of phenotypes in adult male patients with X-linked adrenoleukodystrophy.
Annals of neurology 2001;49(2):186-94.
2000: Hugo W Moser; Daniel J Loes; Elias Melhem; Gerald V Raymond; Lena Bezman; Christiane Sylvia Cox; Shou-En Lu
X-Linked adrenoleukodystrophy: overview and prognosis as a function of age and brain magnetic resonance imaging abnormality. A study involving 372 patients.
Neuropediatrics 2000;31(5):227-39.
2000: MB Lachtermacher; H N Seuanez; Hugo W Moser; Kirby D Smith
One-step multiplex PCR strategy for identification of mutations by SSCP and DNA sequencing.
BioTechniques 2000;29(2):234-6.
2000: Hugo W Moser; Lena Bezman; Shou-En Lu; Gerald V Raymond
Therapy of X-linked adrenoleukodystrophy: prognosis based upon age and MRI abnormality and plans for placebo-controlled trials.
Journal of inherited metabolic disease 2000;23(3):273-7.
2000: Elias Melhem; Daniel J Loes; Christos S Georgiades; Gerald V Raymond; Hugo W Moser
X-linked adrenoleukodystrophy: the role of contrast-enhanced MR imaging in predicting disease progression.
AJNR. American journal of neuroradiology 2000;21(5):839-44.
2000: MB Lachtermacher; H N Seuánez; Ann B Moser; Hugo W Moser; Kirby D Smith
Determination of 30 X-linked adrenoleukodystrophy mutations, including 15 not previously described.
Human mutation 2000;15(4):348-53.
1999: Elias Melhem; Peter B Barker; Gerald V Raymond; Hugo W Moser
X-linked adrenoleukodystrophy in children: review of genetic, clinical, and MR imaging characteristics.
AJR. American journal of roentgenology 1999;173(6):1575-81.
1999: Hugo W Moser
Genotype-phenotype correlations in disorders of peroxisome biogenesis.
Molecular genetics and metabolism 1999;68(2):316-27.
1999: CC Chang; Sarah T South; Daniel S Warren; J Jones; Ann B Moser; Hugo W Moser; Stephen J Gould
Metabolic control of peroxisome abundance.
Journal of cell science 1999;112 ( Pt 10)():1579-90.
1999: Corinne D Boehm; Garry R Cutting; MB Lachtermacher; Hugo W Moser; Samuel S Chong
Accurate DNA-based diagnostic and carrier testing for X-linked adrenoleukodystrophy.
Molecular genetics and metabolism 1999;66(2):128-36.
1999: Ann B Moser; D S Jones; Gerald V Raymond; Hugo W Moser
Plasma and red blood cell fatty acids in peroxisomal disorders.
Neurochemical research 1999;24(2):187-97.
1999: Hugo W Moser
[Therapeutic strategies for peroxisomal disorders].
Revista de neurologia 1999;28 Suppl 1():S65-9.
1999: Hugo W Moser
[Peroxisomal disorders: classification and overview of biochemical abnormalities].
Revista de neurologia 1999;28 Suppl 1():S45-8.
1999: Hugo W Moser
[The peroxisome: structure, function and biogenesis].
Revista de neurologia 1999;28 Suppl 1():S37-9.
1999: Hugo W Moser
Peroxisomal disorders. Molecular defects, genotype-phenotype correlations and therapy.
Le Journal médical libanais. The Lebanese medical journal 1999;47(1):18-21.
1999: Elisabeth G van Grunsven; E van Berkel; Petra A W Mooijer; Paul A Watkins; Hugo W Moser; Yasuyuki Suzuki; L L Jiang; T Hashimoto; Gerald Hoefler; Jerzy Adamski; Ronald J A Wanders
Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.
American journal of human genetics 1999;64(1):99-107.
1999: Ann B Moser; N Kreiter; Lena Bezman; S Lu; Gerald V Raymond; Sakkubai Naidu; Hugo W Moser
Plasma very long chain fatty acids in 3,000 peroxisome disease patients and 29,000 controls.
Annals of neurology 1999;45(1):100-10.
1998: Daniel S Warren; James C Morrell; Hugo W Moser; David Valle; Stephen J Gould
Identification of PEX10, the gene defective in complementation group 7 of the peroxisome-biogenesis disorders.
American journal of human genetics 1998;63(2):347-59.
1998: Lena Bezman; Hugo W Moser
Incidence of X-linked adrenoleukodystrophy and the relative frequency of its phenotypes.
American journal of medical genetics 1998;76(5):415-9.
1998: Hugo W Moser
Neurometabolic disease.
Current opinion in neurology 1998;11(2):91-5.
1998: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Cornelis Jakobs; Hugo W Moser; Ronald J A Wanders
Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.
Clinica chimica acta; international journal of clinical chemistry 1998;271(2):203-11.
1997: BE Reuber; Emily L Germain-Lee; Cynthia S Collins; James C Morrell; R Ameritunga; Hugo W Moser; David Valle; Stephen J Gould
Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders.
Nature genetics 1997;17(4):445-8.
1997: Jyh-Feng Lu; Ann M Lawler; Paul A Watkins; James M Powers; Ann B Moser; Hugo W Moser; Kirby D Smith
A mouse model for X-linked adrenoleukodystrophy.
Proceedings of the National Academy of Sciences of the United States of America 1997;94(17):9366-71.
1997: Hugo W Moser
Adrenoleukodystrophy: phenotype, genetics, pathogenesis and therapy.
Brain : a journal of neurology 1997;120 ( Pt 8)():1485-508.
1997: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Hugo W Moser; Cornelis Jakobs; Hugo S A Heijmans; Ronald J A Wanders
Phytanoyl-CoA hydroxylase is not only deficient in classical Refsum disease but also in rhizomelic chondrodysplasia punctata.
Journal of inherited metabolic disease 1997;20(3):444-6.
1996: Gerbert Jansen; Stephanie J Mihalik; Paul A Watkins; Hugo W Moser; C Jakobs; Simone Denis; R J Wanders
Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans.
Biochemical and biophysical research communications 1996;229(1):205-10.
1996: Hugo W Moser
Peroxisomal disorders.
Seminars in pediatric neurology 1996;3(4):298-304.
1996: David Edwin; LYNN SPEEDIE; W Kohler; Sakkubai Naidu; B Kruse; Hugo W Moser
Cognitive and brain magnetic resonance imaging findings in adrenomyeloneuropathy.
Annals of neurology 1996;40(4):675-8.
1996: Ben Distel; Ralf Erdmann; Stephen J Gould; Günter Blobel; D I Crane; James M Cregg; Gabriele Dodt; Y Fujiki; J M Goodman; W W Just; Jan A K W Kiel; Wolf-H Kunau; Paul B LAZAROW; Guy P Mannaerts; Hugo W Moser; T Osumi; R A Rachubinski; A Roscher; Suresh Subramani; Henk F Tabak; T Tsukamoto; David Valle; Ida Van der klei; P P van Veldhoven; M Veenhuis
A unified nomenclature for peroxisome biogenesis factors.
The Journal of cell biology 1996;135(1):1-3.
1996: Vinay Chaudhry; Hugo W Moser; David R Cornblath
Nerve conduction studies in adrenomyeloneuropathy.
Journal of neurology, neurosurgery, and psychiatry 1996;61(2):181-5.
1996: T Yahraus; Nancy Braverman; Gabriele Dodt; J E Kalish; James C Morrell; Hugo W Moser; David Valle; Stephen J Gould
The peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptor.
The EMBO journal 1996;15(12):2914-23.
1996: Elias Melhem; S N Breiter; Aziz Ulug; Gerald V Raymond; Hugo W Moser
Improved tissue characterization in adrenoleukodystrophy using magnetization transfer imaging.
AJR. American journal of roentgenology 1996;166(3):689-95.
1996: Walter E Kaufmann; CHRISTIANE THEDA; Sakkubai Naidu; Paul A Watkins; Ann B Moser; Hugo W Moser
Neuronal migration abnormality in peroxisomal bifunctional enzyme defect.
Annals of neurology 1996;39(2):268-71.
1995: Paul A Watkins; Martina C McGuinness; Gerald V Raymond; B A Hicks; Jeanne Sisk; Ann B Moser; Hugo W Moser
Distinction between peroxisomal bifunctional enzyme and acyl-CoA oxidase deficiencies.
Annals of neurology 1995;38(3):472-7.
1995: Hugo W Moser
Clinical and therapeutic aspects of adrenoleukodystrophy and adrenomyeloneuropathy.
Journal of neuropathology and experimental neurology 1995;54(5):740-5.
1995: Martina C McGuinness; Diane E Griffin; Gerald V Raymond; C A Washington; Hugo W Moser; Kirby D Smith
Tumor necrosis factor-alpha and X-linked adrenoleukodystrophy.
Journal of neuroimmunology 1995;61(2):161-9.
1995: Paul A Watkins; Stephen J Gould; M A Smith; Lelita T Braiterman; H M Wei; F Kok; Ann B Moser; Hugo W Moser; Kirby D Smith
Altered expression of ALDP in X-linked adrenoleukodystrophy.
American journal of human genetics 1995;57(2):292-301.
1995: Hugo W Moser; J M Powers; Kirby D Smith
Adrenoleukodystrophy: molecular genetics, pathology, and Lorenzo's oil.
Brain pathology (Zurich, Switzerland) 1995;5(3):259-66.
1995: Arvind Kumar; W Köhler; B Kruse; Sakkubai Naidu; A Bergin; David Edwin; Hugo W Moser
MR findings in adult-onset adrenoleukodystrophy.
AJNR. American journal of neuroradiology 1995;16(6):1227-37.
1995: Hugo W Moser
Adrenoleukodystrophy.
Current opinion in neurology 1995;8(3):221-6.
1995: M L Slawecki; G Dodt; Steven J Steinberg; Ann B Moser; Hugo W Moser; Stephen J Gould
Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders.
Journal of cell science 1995;108 ( Pt 5)():1817-29.
1995: Gabriele Dodt; Nancy Braverman; C Wong; Ann B Moser; Hugo W Moser; Paul A Watkins; David Valle; Stephen J Gould
Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders.
Nature genetics 1995;9(2):115-25.
1995: Hugo W Moser
Komrower Lecture. Adrenoleukodystrophy: natural history, treatment and outcome.
Journal of inherited metabolic disease 1995;18(4):435-47.
1994: Sakkubai Naidu; Hugo W Moser
Peroxisomal disorders.
Neurologic clinics 1994;12(4):727-39.
1994: B Kruse; Peter B Barker; Peter C M van Zijl; Jeff H Duyn; C T Moonen; Hugo W Moser
Multislice proton magnetic resonance spectroscopic imaging in X-linked adrenoleukodystrophy.
Annals of neurology 1994;36(4):595-608.
1994: M Rasmussen; Ann B Moser; J Borel; S Khangoora; Hugo W Moser
Brain, liver, and adipose tissue erucic and very long chain fatty acid levels in adrenoleukodystrophy patients treated with glyceryl trierucate and trioleate oils (Lorenzo's oil).
Neurochemical research 1994;19(8):1073-82.
1994: L S Blevins; J Shankroff; Hugo W Moser; Paul W Ladenson
Elevated plasma adrenocorticotropin concentration as evidence of limited adrenocortical reserve in patients with adrenomyeloneuropathy.
The Journal of clinical endocrinology and metabolism 1994;78(2):261-5.
1993: M Alpern; GEORGE SACK; D H Krantz; J Jenness; H Zhang; Hugo W Moser
Chromosomal rearrangement segregating with adrenoleukodystrophy: associated changes in color vision.
Proceedings of the National Academy of Sciences of the United States of America 1993;90(20):9494-8.
1993: GEORGE SACK; M Alpern; T Webster; Robert Feil; James C Morrell; G Chen; W Chen; C T Caskey; Hugo W Moser
Chromosomal rearrangement segregating with adrenoleukodystrophy: a molecular analysis.
Proceedings of the National Academy of Sciences of the United States of America 1993;90(20):9489-93.
1993: M J Santos; Ann B Moser; H Drwinga; Hugo W Moser; Paul B LAZAROW
Analysis of peroxisomes in lymphoblasts: Zellweger syndrome and a patient with a deletion in chromosome 7.
Pediatric research 1993;33(5):441-4.
1993: CHRISTIANE THEDA; R C Woody; Sakkubai Naidu; Ann B Moser; Hugo W Moser
Increased very long chain fatty acids in patients on a ketogenic diet: a cause of diagnostic confusion.
The Journal of pediatrics 1993;122(5 Pt 1):724-6.
1993: W H Zinkham; Thomas S Kickler; J Borel; Hugo W Moser
Lorenzo's oil and thrombocytopenia in patients with adrenoleukodystrophy.
The New England journal of medicine 1993;328(15):1126-7.
1993: Hugo W Moser
Lorenzo's oil.
Lancet 1993;341(8844):544.
1993: Hugo W Moser
Peroxisomal diseases.
Advances in human genetics 1993;21():1-106, 443-51.
1993: S Stöckler; MM Millner; Brunhilde Molzer; Franz Ebner; E Körner; Hugo W Moser
Multiple sclerosis-like syndrome in a woman heterozygous for adrenoleukodystrophy.
European neurology 1993;33(5):390-2.
1992: CHRISTIANE THEDA; Ann B Moser; J M Powers; Hugo W Moser
Phospholipids in X-linked adrenoleukodystrophy white matter: fatty acid abnormalities before the onset of demyelination.
Journal of the neurological sciences 1992;110(1-2):195-204.
1992: Hugo W Moser
New concepts in the diagnosis and treatment of lysosomal and peroxisomal disorders.
Current opinion in neurology and neurosurgery 1992;5(3):355-8.
1992: Raphael A Zoeller; S Rangaswamy; H Herscovitz; W B Rizzo; Amiya K Hajra; Arun K Das; Hugo W Moser; Ann B Moser; Paul B LAZAROW; M J Santos
Mutants in a macrophage-like cell line are defective in plasmalogen biosynthesis, but contain functional peroxisomes.
The Journal of biological chemistry 1992;267(12):8299-306.
1992: M J Santos; S Hoefler; Ann B Moser; Hugo W Moser; Paul B LAZAROW
Peroxisome assembly mutations in humans: structural heterogeneity in Zellweger syndrome.
Journal of cellular physiology 1992;151(1):103-12.
1992: Hugo W Moser; Ann B Moser; Kirby D Smith; A Bergin; J Borel; J Shankroff; O Colin Stine; C Merette; J Ott; W Krivit
Adrenoleukodystrophy: phenotypic variability and implications for therapy.
Journal of inherited metabolic disease 1992;15(4):645-64.
1991: Gerald Hoefler; Eduard Paschke; S Hoefler; Ann B Moser; Hugo W Moser
Photosensitized killing of cultured fibroblasts from patients with peroxisomal disorders due to pyrene fatty acid-mediated ultraviolet damage.
The Journal of clinical investigation 1991;88(6):1873-9.
1991: J Aikawa; W W Chen; Richard I Kelley; K Tada; Hugo W Moser; G L Chen
Low-density particles (W-particles) containing catalase in Zellweger syndrome and normal fibroblasts.
Proceedings of the National Academy of Sciences of the United States of America 1991;88(22):10084-8.
1991: Sakkubai Naidu; Hugo W Moser
Value of neuroimaging in metabolic diseases affecting the CNS.
AJNR. American journal of neuroradiology 1991;12(3):413-6.
1991: Jutta Gärtner; W W Chen; Richard I Kelley; Stephanie J Mihalik; Hugo W Moser
The 22-kD peroxisomal integral membrane protein in Zellweger syndrome--presence, abundance, and association with a peroxisomal thiolase precursor protein.
Pediatric research 1991;29(2):141-6.
1990: Martina C McGuinness; Ann B Moser; Hugo W Moser; Paul A Watkins
Peroxisomal disorders: complementation analysis using beta-oxidation of very long chain fatty acids.
Biochemical and biophysical research communications 1990;172(1):364-9.
1990: Sakkubai Naidu; Hugo W Moser
Peroxisomal disorders.
Neurologic clinics 1990;8(3):507-28.
1990: Hugo W Moser; Ann B Moser; W W Chen; Paul A Watkins
Adrenoleukodystrophy and Zellweger syndrome.
Progress in clinical and biological research 1990;321():511-35.
1990: Jutta Gärtner; A Balfe; W W Chen; Hugo W Moser
Study of peroxisomal proteins in patients with Zellweger syndrome.
Journal of inherited metabolic disease 1990;13(3):383-6.
1989: B J Al; CAROL TIFFANY; D S Gomes de Mesquita; Hugo W Moser; J M Tager; A W Schram
Properties of acid ceramidase from human spleen.
Biochimica et biophysica acta 1989;1004(2):245-51.
1989: GEORGE SACK; M B Raven; Hugo W Moser
Color vision defects in adrenomyeloneuropathy.
American journal of human genetics 1989;44(6):794-8.
1989: Stephanie J Mihalik; Hugo W Moser; Paul A Watkins; D M Danks; A Poulos; W J Rhead
Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients.
Pediatric research 1989;25(5):548-52.
1989: Hugo W Moser
Peroxisomal diseases.
Advances in pediatrics 1989;36():1-38.
1989: Hugo W Moser; Stephanie J Mihalik; Paul A Watkins
Adrenoleukodystrophy and other peroxisomal disorders that affect the nervous system, including new observations on L-pipecolic acid oxidase in primates.
Brain & development 1989;11(2):80-90.
1988: S Hoefler; Gerald Hoefler; Ann B Moser; Paul A Watkins; W W Chen; Hugo W Moser
Prenatal diagnosis of rhizomelic chondrodysplasia punctata.
Prenatal diagnosis 1988;8(8):571-6.
1988: H Shimizu; Hugo W Moser; Sakkubai Naidu
Auditory brainstem response and audiologic findings in adrenoleukodystrophy: its variant and carrier.
Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery 1988;98(3):215-20.
1988: P R Aubourg; GEORGE SACK; Hugo W Moser
Frequent alterations of visual pigment genes in adrenoleukodystrophy.
American journal of human genetics 1988;42(3):408-13.
1988: G M Small; M J Santos; T Imanaka; A Poulos; D M Danks; Hugo W Moser; Paul B LAZAROW
Peroxisomal integral membrane proteins in livers of patients with Zellweger syndrome, infantile Refsum's disease and X-linked adrenoleukodystrophy.
Journal of inherited metabolic disease 1988;11(4):358-71.
1987: P R Aubourg; GEORGE SACK; Deborah A Meyers; J J Lease; Hugo W Moser
Linkage of adrenoleukodystrophy to a polymorphic DNA probe.
Annals of neurology 1987;21(4):349-52.
1987: Ann B Moser; J Borel; A Odone; Sakkubai Naidu; David R Cornblath; D B Sanders; Hugo W Moser
A new dietary therapy for adrenoleukodystrophy: biochemical and preliminary clinical results in 36 patients.
Annals of neurology 1987;21(3):240-9.
1987: S J Qualman; Hugo W Moser; David Valle; A E Moser; S E Antonarakis; J K Boitnott; W H Zinkham
Farber disease: pathologic diagnosis in sibs with phenotypic variability.
American journal of medical genetics. Supplement 1987;3():233-41.
1984: S E Antonarakis; David Valle; Hugo W Moser; Ann B Moser; S J Qualman; W H Zinkham
Phenotypic variability in siblings with Farber disease.
The Journal of pediatrics 1984;104(3):406-9.