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Pseudo-Zellweger syndrome

Also Known As:
Pseudo Zellweger syndrome leukodystrophy
Networked: 11 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Related Diseases

1. Peroxisomal Disorders (Peroxisomal Disorder)
01/01/1995 - "Other peroxisomal disorders (pseudo-Zellweger syndrome, neonatal adrenoleukodystrophy (NALD), pseudo-neonatal adrenoleukodystrophy, rhizomelic chondrodysplasia punctata (RCDP), and hyperpipecolic acidaemia) share some of these symptoms, but with varying organ involvement, severity of dysfunction, and duration of survival. "
01/01/1990 - "Electron microscopy and cytochemical staining for catalase were used to identify peroxisomes in two boys with infantile Refsum's disease (IRD), a girl with autopsy confirmed neonatal adrenoleukodystrophy (NALD), and two boys with pseudo-Zellweger syndrome (PZS). "
02/01/1994 - "From the biochemical point some diseases present alterations of the cholesterol side chain (Zellweger syndrome, pseudo-Zellweger syndrome, infantile Refsum's disease, neonatal adrenoleukodystrophy), other diseases present errors involving the steroid nucleus (familial giant cell hepatitis). "
03/31/1988 - "Peroxisomal anomalities are central features of Zellweger's cerebro-hepato-renal syndrome, neonatal adrenoleukodystrophy, infantile Refsum's disease and several other genetic metabolic disorders (pseudo-Zellweger syndrome, Leber congenital amaurosis, cerebrotendinous xanthomatosis, rhizomelic chondrodysplasia punctata). "
01/01/1989 - "Peroxisomal disorders occur as two main groups: 1/ disorders with multiple deficiencies of peroxisomal functions: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease, rhizomelic chondrodysplasia punctata; 2/ disorders with a single peroxisomal enzyme defect: X-linked adrenoleukodystrophy, acatalasemia, type 1 hyperoxaluria, pseudo-Zellweger syndrome. "
2. Zellweger Syndrome (Zellweger's Syndrome)
04/01/1993 - "One patient with Zellweger syndrome had a unique finding of a positive granular catalase reaction and a negative reaction with antisera to 3-ketoacyl-coenzyme A thiolase, which suggested a diagnosis of pseudo-Zellweger syndrome. "
01/01/1989 - "The activation of palmitate and trihydroxycoprostanic acid and the peroxisomal oxidation of palmitate, trihydroxycoprostanic acid and their CoA esters were measured in homogenates prepared from fresh liver tissue of patients undergoing hepatic surgery and from frozen postmortem liver specimens of controls, patients with Zellweger syndrome and a patient with pseudo-Zellweger syndrome, a deficiency of peroxisomal 3-oxoacyl-CoA thiolase. "
02/01/1994 - "From the biochemical point some diseases present alterations of the cholesterol side chain (Zellweger syndrome, pseudo-Zellweger syndrome, infantile Refsum's disease, neonatal adrenoleukodystrophy), other diseases present errors involving the steroid nucleus (familial giant cell hepatitis). "
03/31/1988 - "Peroxisomal anomalities are central features of Zellweger's cerebro-hepato-renal syndrome, neonatal adrenoleukodystrophy, infantile Refsum's disease and several other genetic metabolic disorders (pseudo-Zellweger syndrome, Leber congenital amaurosis, cerebrotendinous xanthomatosis, rhizomelic chondrodysplasia punctata). "
01/01/1989 - "Peroxisomal disorders occur as two main groups: 1/ disorders with multiple deficiencies of peroxisomal functions: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease, rhizomelic chondrodysplasia punctata; 2/ disorders with a single peroxisomal enzyme defect: X-linked adrenoleukodystrophy, acatalasemia, type 1 hyperoxaluria, pseudo-Zellweger syndrome. "
3. Infantile Refsum Disease (Infantile Phytanic Acid Storage Disease)
01/01/1990 - "Electron microscopy and cytochemical staining for catalase were used to identify peroxisomes in two boys with infantile Refsum's disease (IRD), a girl with autopsy confirmed neonatal adrenoleukodystrophy (NALD), and two boys with pseudo-Zellweger syndrome (PZS). "
02/01/1994 - "From the biochemical point some diseases present alterations of the cholesterol side chain (Zellweger syndrome, pseudo-Zellweger syndrome, infantile Refsum's disease, neonatal adrenoleukodystrophy), other diseases present errors involving the steroid nucleus (familial giant cell hepatitis). "
03/31/1988 - "Peroxisomal anomalities are central features of Zellweger's cerebro-hepato-renal syndrome, neonatal adrenoleukodystrophy, infantile Refsum's disease and several other genetic metabolic disorders (pseudo-Zellweger syndrome, Leber congenital amaurosis, cerebrotendinous xanthomatosis, rhizomelic chondrodysplasia punctata). "
01/01/1989 - "Peroxisomal disorders occur as two main groups: 1/ disorders with multiple deficiencies of peroxisomal functions: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease, rhizomelic chondrodysplasia punctata; 2/ disorders with a single peroxisomal enzyme defect: X-linked adrenoleukodystrophy, acatalasemia, type 1 hyperoxaluria, pseudo-Zellweger syndrome. "
12/01/1988 - "These diseases include: (1) those in which peroxisomes are virtually absent leading to a generalized impairment of peroxisomal functions (the cerebro-hepato-renal syndrome of Zellweger, neonatal adrenoleukodystrophy, infantile Refsum disease and hyperpipecolic acidaemia); (2) those in which peroxisomes are present and several peroxisomal functions are impaired (the rhizomelic form of chondrodysplasia punctata, combined peroxisomal beta-oxidation enzyme protein deficiency); and (3) those in which peroxisomes are present and only a single peroxisomal function is impaired (X-linked adrenoleukodystrophy, peroxisomal thiolase deficiency (pseudo-Zellweger syndrome), acyl-CoA oxidase deficiency (pseudo-neonatal adrenoleukodystrophy) and probably, the classic form of Refsum disease."
4. Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
03/01/1988 - "The clinical, biochemical, and cytochemical features found in these two siblings are compared with those seen in two other disorders characterized by the absence of a decreased number of hepatic peroxisomes and the presence of VLCFA: (1) pseudo-Zellweger syndrome (deficiency of peroxisomal thiolase activity) and (2) X-linked childhood adrenoleukodystrophy (deficiency of activation of lignoceric acid). "
01/01/1989 - "Peroxisomal disorders occur as two main groups: 1/ disorders with multiple deficiencies of peroxisomal functions: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease, rhizomelic chondrodysplasia punctata; 2/ disorders with a single peroxisomal enzyme defect: X-linked adrenoleukodystrophy, acatalasemia, type 1 hyperoxaluria, pseudo-Zellweger syndrome. "
06/15/1987 - "Such diseases include X-linked adrenoleukodystrophy (peroxisomal very long chain fatty acyl CoA ligase deficiency), 'pseudo-Zellweger syndrome' (peroxisomal 3-oxoacyl-CoA thiolase deficiency) and 'pseudo-neonatal adrenoleukodystrophy' (acyl-CoA oxidase deficiency)."
12/01/1988 - "These diseases include: (1) those in which peroxisomes are virtually absent leading to a generalized impairment of peroxisomal functions (the cerebro-hepato-renal syndrome of Zellweger, neonatal adrenoleukodystrophy, infantile Refsum disease and hyperpipecolic acidaemia); (2) those in which peroxisomes are present and several peroxisomal functions are impaired (the rhizomelic form of chondrodysplasia punctata, combined peroxisomal beta-oxidation enzyme protein deficiency); and (3) those in which peroxisomes are present and only a single peroxisomal function is impaired (X-linked adrenoleukodystrophy, peroxisomal thiolase deficiency (pseudo-Zellweger syndrome), acyl-CoA oxidase deficiency (pseudo-neonatal adrenoleukodystrophy) and probably, the classic form of Refsum disease."
5. Rhizomelic Chondrodysplasia Punctata

Related Drugs and Biologics

1. Acyl-CoA Oxidase
2. Coenzyme A (CoA)
3. Catalase
4. Immune Sera (Antisera)
5. Esters
6. Cholesterol
7. Acyl Coenzyme A (Acyl CoA)
8. lignoceric acid (tetracosanoic acid)