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Peroxisomal Disorders (Peroxisomal Disorder)

A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include ZELLWEGER SYNDROME; INFANTILE REFSUM DISEASE; rhizomelic chondrodysplasia (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and ADRENOLEUKODYSTROPHY (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders.
Also Known As:
Peroxisomal Disorder; Adrenoleukodystrophy, Autosomal, Neonatal Form; Peroxisomal Dysfunction, General; Peroxisomal Dysfunction, Multiple; Peroxisomal Dysfunction, Single; Acidemia, Hyperpipecolic; Acidemias, Hyperpipecolic; Adrenoleukodystrophies, Neonatal; Dysfunction, General Peroxisomal; Dysfunction, Multiple Peroxisomal; Dysfunction, Single Peroxisomal; Dysfunctions, General Peroxisomal; Dysfunctions, Multiple Peroxisomal; Dysfunctions, Single Peroxisomal; General Peroxisomal Dysfunction; General Peroxisomal Dysfunctions; Hyperpipecolic Acidemias; Multiple Peroxisomal Dysfunction; Multiple Peroxisomal Dysfunctions; Neonatal Adrenoleukodystrophies; Neonatal Adrenoleukodystrophy; Peroxisomal Dysfunctions, General; Peroxisomal Dysfunctions, Multiple; Peroxisomal Dysfunctions, Single; Single Peroxisomal Dysfunction; Single Peroxisomal Dysfunctions; Adrenoleukodystrophy, Neonatal; Hyperpipecolic Acidemia
Networked: 360 relevant articles (2 outcomes, 17 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Zellweger Syndrome (Zellweger's Syndrome)
2. Multiple Sulfatase Deficiency Disease (Mucosulfatidosis)
3. Ichthyosis (Xeroderma)
4. Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
5. Infantile Refsum Disease (Infantile Phytanic Acid Storage Disease)

Experts

1. Wanders, Ronald J A: 13 articles (12/2012 - 02/2003)
2. Wajner, Moacir: 7 articles (06/2013 - 09/2006)
3. Shimozawa, N: 6 articles (07/2001 - 01/2000)
4. Berger, Johannes: 5 articles (01/2015 - 06/2012)
5. Busanello, Estela Natacha Brandt: 5 articles (06/2013 - 09/2010)
6. Tonin, Anelise Miotti: 5 articles (06/2013 - 09/2010)
7. Ferdinandusse, Sacha: 5 articles (12/2012 - 08/2003)
8. Waterham, Hans R: 5 articles (04/2012 - 02/2003)
9. Shimozawa, Nobuyuki: 5 articles (04/2012 - 10/2003)
10. Wanders, R J A: 5 articles (04/2008 - 01/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Peroxisomal Disorders:
1. Fatty Acids (Saturated Fatty Acids)IBA
2. alkylglycerone-phosphate synthaseIBA
3. Phytanic AcidIBA
4. pristanic acidIBA
5. EnzymesIBA
6. pipecolic acidIBA
7. CatalaseIBA
8. Immune Sera (Antisera)IBA
9. Acyl-CoA OxidaseIBA
10. LipidsIBA

Therapies and Procedures

1. Ketogenic Diet
2. Cell Transplantation
3. Bone Marrow Transplantation (Transplantation, Bone Marrow)
4. Drug Therapy (Chemotherapy)