Striatonigral degeneration infantile

Also Known As:

Bilateral Striatal Necrosis, Infantile; Infantile bilateral striatal necrosis; Striatal Degeneration, Familial; Striatal degeneration familial; Striatonigral Degeneration, Infantile

Networked: 3 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Nervous System Diseases: 14178
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Basal Ganglia Diseases: 346
      - Multiple System Atrophy: 1925
        Striatonigral Degeneration: 94
        Striatonigral degeneration infantile: 3
  - Movement Disorders: 4634
    - Multiple System Atrophy: 1925
      - Striatonigral Degeneration: 94
        Striatonigral degeneration infantile: 3
- Autonomic Nervous System Diseases: 722
  - Primary Dysautonomias: 5
    - Multiple System Atrophy: 1925
      - Striatonigral Degeneration: 94
        Striatonigral degeneration infantile: 3

Experts

1.	Chien, Man-Ling: 1 article (11/2020)
2.	Juang, Yue-Li: 1 article (11/2020)
3.	Lai, Jian-Han: 1 article (11/2020)
4.	Lin, Ting-Fong: 1 article (11/2020)
5.	Yang, Wan-Syuan: 1 article (11/2020)
6.	Bessler, Hanna: 1 article (01/2017)
7.	Djaldetti, Meir: 1 article (01/2017)
8.	Ganelin Cohen, Esther: 1 article (01/2017)
9.	Straussberg, Rachel: 1 article (01/2017)
10.	Amir, J: 1 article (10/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Striatonigral degeneration infantile:

1.	Mutant Proteins (Protein, Mutant)IBA 11/01/2020 - "Furthermore, overexpression of NUP62Q391P, a mutant protein causing autosomal recessive infantile bilateral striatal necrosis, resulted in a defect in the SAC, indicating that the amino acid residue Q391 in NUP62 is crucial for its effect on the SAC. "
2.	CytokinesIBA 01/01/2017 - "Cytokine Production by Mononuclear Cells from Patients with Familial Infantile Bilateral Striatal Necrosis." 01/01/2017 - "Prompted by findings suggesting immune instability in infantile bilateral striatal necrosis (IBSN), we evaluated levels of proinflammatory (tumor necrosis factor α, interleukin [IL]-1β, IL-6, interferon [IFN]γ) and anti-inflammatory (IL-10 and IL-1ra) cytokines produced by peripheral blood mononuclear cells (PBMC) from 6 children with IBSN and 11 age-matched controls. "
3.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 01/01/2017 - "Prompted by findings suggesting immune instability in infantile bilateral striatal necrosis (IBSN), we evaluated levels of proinflammatory (tumor necrosis factor α, interleukin [IL]-1β, IL-6, interferon [IFN]γ) and anti-inflammatory (IL-10 and IL-1ra) cytokines produced by peripheral blood mononuclear cells (PBMC) from 6 children with IBSN and 11 age-matched controls. "
4.	InterleukinsIBA 01/01/2017 - "Prompted by findings suggesting immune instability in infantile bilateral striatal necrosis (IBSN), we evaluated levels of proinflammatory (tumor necrosis factor α, interleukin [IL]-1β, IL-6, interferon [IFN]γ) and anti-inflammatory (IL-10 and IL-1ra) cytokines produced by peripheral blood mononuclear cells (PBMC) from 6 children with IBSN and 11 age-matched controls. "
5.	InterferonsIBA 01/01/2017 - "Prompted by findings suggesting immune instability in infantile bilateral striatal necrosis (IBSN), we evaluated levels of proinflammatory (tumor necrosis factor α, interleukin [IL]-1β, IL-6, interferon [IFN]γ) and anti-inflammatory (IL-10 and IL-1ra) cytokines produced by peripheral blood mononuclear cells (PBMC) from 6 children with IBSN and 11 age-matched controls. "
6.	Biotin (Vitamin H)FDA Link 10/08/2002 - "Familial infantile bilateral striatal necrosis: clinical features and response to biotin treatment."
7.	Amino Acids FDA Link 11/01/2020 - "Furthermore, overexpression of NUP62Q391P, a mutant protein causing autosomal recessive infantile bilateral striatal necrosis, resulted in a defect in the SAC, indicating that the amino acid residue Q391 in NUP62 is crucial for its effect on the SAC. "