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Tiglic acidemia

Also Known As:

Disorder of isoleucine metabolism

Networked: 4 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Amino Acid Metabolism: 1
      - Tiglic acidemia: 4
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Amino Acid Metabolism: 1
      - Tiglic acidemia: 4

Related Diseases

1.	2-Methylbutyryl-CoA Dehydrogenase Deficiency

Experts

1.	Alvorcem, Leonardo de Moura: 1 article (10/2012)
2.	Grings, Mateus: 1 article (10/2012)
3.	Knebel, Lisiane Aurélio: 1 article (10/2012)
4.	Leipnitz, Guilhian: 1 article (10/2012)
5.	Tonin, Anelise Miotti: 1 article (10/2012)
6.	Wajner, Moacir: 1 article (10/2012)
7.	Zanatta, Ângela: 1 article (10/2012)
8.	Alfardan, Jaffar: 1 article (08/2010)
9.	Copeland, Sara: 1 article (08/2010)
10.	Ellison, Jay: 1 article (08/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Tiglic acidemia:

1.	2-methylacyl-CoA dehydrogenaseIBA 08/01/2010 - "Short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency, also known as 2-methylbutyryl-CoA dehydrogenase deficiency, is a recently described autosomal recessive disorder of isoleucine metabolism. " 10/10/2012 - "Short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency is an autosomal recessive disorder of isoleucine metabolism biochemically characterized by accumulation of 2-methylbutyrylglycine (2MBG) and 2-methylbutyric acid (2MB). "
2.	Mitochondrial DNA (mtDNA)IBA 10/22/2022 - "HSD10 disease was originally described as a disorder of isoleucine metabolism, but the clinical manifestations were subsequently shown to be linked to impaired mtDNA transcript processing due to deficient function of HSD10 in the mtRNase P complex. "
3.	2-methyl-3-hydroxybutyryl-coenzyme A dehydrogenase (2-methyl-3-hydroxybutyryl-CoA dehydrogenase)IBA 01/01/2004 - "We describe a further case of recently reported 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency, a disorder of isoleucine metabolism. "
4.	2-methylbutanoic acidIBA 10/10/2012 - "Short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency is an autosomal recessive disorder of isoleucine metabolism biochemically characterized by accumulation of 2-methylbutyrylglycine (2MBG) and 2-methylbutyric acid (2MB). "
5.	2-methylbutyrylglycineIBA 10/10/2012 - "Short/branched chain acyl-CoA dehydrogenase (SBCAD) deficiency is an autosomal recessive disorder of isoleucine metabolism biochemically characterized by accumulation of 2-methylbutyrylglycine (2MBG) and 2-methylbutyric acid (2MB). "