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Pseudo-Zellweger syndrome
Also Known As:
Pseudo Zellweger syndrome leukodystrophy
Networked:
10
relevant articles (
0
outcomes,
1
trials/studies)
Disease Context: Research Results
Digestive System Diseases: 1042
Liver Diseases: 41660
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Nervous System Diseases: 14178
Central Nervous System Diseases: 4213
Brain Diseases: 15694
Metabolic Brain Diseases: 215
Inborn Metabolic Brain Diseases: 2
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Urogenital Diseases: 126
Urologic Diseases: 799
Kidney Diseases: 12250
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Female Urogenital Diseases and Pregnancy Complications
Female Urogenital Diseases
Urologic Diseases: 799
Kidney Diseases: 12250
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Male Urogenital Diseases
Urologic Diseases: 799
Kidney Diseases: 12250
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
Congenital Abnormalities: 25723
Multiple Abnormalities: 211
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Inborn Genetic Diseases: 11939
Inborn Errors Metabolism: 1162
Peroxisomal Disorders: 504
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Inborn Metabolic Brain Diseases: 2
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Nutritional and Metabolic Diseases: 15
Metabolic Diseases: 10142
Inborn Errors Metabolism: 1162
Peroxisomal Disorders: 504
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Inborn Metabolic Brain Diseases: 2
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Metabolic Brain Diseases: 215
Inborn Metabolic Brain Diseases: 2
Zellweger Syndrome: 511
Pseudo-Zellweger syndrome: 10
Related Diseases
1.
Peroxisomal Disorders (Peroxisomal Disorder)
2.
Zellweger Syndrome (Zellweger's Syndrome)
3.
Infantile Refsum Disease (Infantile Phytanic Acid Storage Disease)
4.
Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
5.
Acatalasia (Acatalasemia)
Drugs and Biologics
Drugs and Important Biological Agents (IBA) related to Pseudo-Zellweger syndrome:
1.
Enzymes
IBA
11/01/1993 - "
A cell fusion study suggested that the enzyme responsible for this case of 'pseudo-Zellweger syndrome' is bifunctional.
"
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.
"
11/01/1993 - "
A case of pseudo-Zellweger syndrome with a possible bifunctional enzyme deficiency but detectable enzyme protein.
"
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.
"
2.
Acyl-CoA Oxidase
IBA
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.
"
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).
"
3.
Coenzyme A (CoA)
IBA
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.
"
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).
"
4.
Catalase
IBA
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).
"
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.
"
5.
Steroids
IBA
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).
"
6.
Proteins (Proteins, Gene)
FDA Link
11/01/1993 - "
A case of pseudo-Zellweger syndrome with a possible bifunctional enzyme deficiency but detectable enzyme protein.
"
7.
Palmitates
IBA
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.
"
8.
Ligases (Synthetase)
IBA
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).
"
9.
Immune Sera (Antisera)
IBA
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.
"
10.
Esters
IBA
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.
"