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3-hydroxyisovalerylcarnitine

Also Known As:

3-HIVA-carnitine

Networked: 8 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Organic Chemicals: 133
- Amines: 4871
  - Quaternary Ammonium Compounds: 136
    - Trimethyl Ammonium Compounds: 1
      - Carnitine: 4291
        3-hydroxyisovalerylcarnitine: 8

Experts

1.	Bektaş, Ömer: 1 article (10/2021)
2.	Ceylan, Ahmet Cevdet: 1 article (10/2021)
3.	Sürücü Kara, İlknur: 1 article (10/2021)
4.	Teber, Serap: 1 article (10/2021)
5.	Yıldırım, Miraç: 1 article (10/2021)
6.	Şahin, Süleyman: 1 article (10/2021)
7.	Ishige, Nobuyuki: 1 article (07/2021)
8.	Nakajima, Hideki: 1 article (07/2021)
9.	Shigematsu, Yosuke: 1 article (07/2021)
10.	Tajima, Go: 1 article (07/2021)

Related Diseases

1.	Biotin deficiency 01/01/2015 - "Surprisingly, studies indicate that increased urinary 3-hydroxyisovalerylcarnitine (3HIAc), a previously validated marker of biotin deficiency, is not a valid biomarker in pregnancy. " 05/01/2013 - "Increased 3-hydroxyisovalerylcarnitine (C5OH) is regarded as a biomarker of biotin deficiency. "
2.	Multiple Carboxylase Deficiency (Deficiency, Multiple Carboxylase) 05/01/1993 - "Identification of 3-hydroxyisovalerylcarnitine in the urine of a patient with multiple carboxylase deficiency." 07/15/2008 - "Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency."
3.	Biotinidase Deficiency (Multiple Carboxylase Deficiency, Late Onset) 12/01/2009 - "(1) Four patients with biotinidase deficiency were diagnosed by characteristic metabolites, such as elevated blood levels of 3-hydroxyisovalerylcarnitine (6.22 +/- 3.1 mumol/L), elevated 3-methylcrontonylglycine, methylcitrate and 3-hydroxypropionate in urine and very low biotinidase activities. "
4.	Inborn Errors Metabolism (Inborn Errors of Metabolism) 01/01/2003 - "Since the addition of tandem mass spectrometry (MS/MS) to the North Carolina Newborn Screening Program, 20 infants with two consecutive elevated 3-hydroxyisovalerylcarnitine (C5OH) levels have been evaluated for evidence of inborn errors of metabolism associated with this metabolite. "
5.	Atrophy 10/01/2021 - "The detailed biochemical and metabolic tests were unremarkable, except blood 3-hydroxyisovalerylcarnitine (C5OH) was slightly increased in case 1. Cranial computed tomography demonstrated mild cerebral and cerebellar atrophy as well as bilateral periventricular and thalamic calcifications in both cases. "

Related Drugs and Biologics

1.	Biomarkers (Surrogate Marker)
2.	Biotinidase
3.	3-methylcrotonyl CoA carboxylase 1 deficiency
4.	acylcarnitine
5.	3-hydroxyglutaric acid
6.	hydracrylic acid
7.	beta-hydroxyisovaleric acid (beta-hydroxy-beta-methylbutyrate)