HOME PRODUCTS COMPANY CONTACT FAQ

Research Dictionary Pharma

Sign Up FREE or Login

X-linked recessive Bulbospinal neuronopathy

Also Known As:

Bulbospinal neuronopathy, X-linked recessive

Networked: 9 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Nervous System Diseases: 14178
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Nervous System Heredodegenerative Disorders: 63
    - X-Linked Bulbo-Spinal Atrophy: 541
      - X-linked recessive Bulbospinal neuronopathy: 9
  - X-Linked Genetic Diseases: 42
    - X-Linked Bulbo-Spinal Atrophy: 541
      - X-linked recessive Bulbospinal neuronopathy: 9

Related Diseases

1.	X-Linked Bulbo-Spinal Atrophy
2.	Multiple System Atrophy
3.	Gynecomastia
4.	Atrophy
5.	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)

Experts

1.	Pradat, P-F: 1 article (05/2017)
2.	Querin, G: 1 article (05/2017)
3.	Sorarù, G: 1 article (05/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to X-linked recessive Bulbospinal neuronopathy:

1.	Androgen Receptors (Androgen Receptor)IBA 12/01/1995 - "X-linked recessive bulbospinal neuronopathy (SBMA) is an adult onset motor neuronopathy with androgen receptor (AR) gene mutation of expanded CAG repeat size in the first exon. " 12/01/1995 - "Clinical phenotypes and the CAG repeat size of the androgen receptor gene were assessed in 95 Japanese patients with X-linked recessive bulbospinal neuronopathy. " 12/01/1995 - "X-linked recessive bulbospinal neuronopathy: clinical phenotypes and CAG repeat size in androgen receptor gene." 07/01/1993 - "Very late onset X-linked recessive bulbospinal neuronopathy: mild clinical features and a mild increase in the size of tandem CAG repeat in androgen receptor gene." 11/01/1992 - "The genetic mutation of X-linked recessive bulbospinal neuronopathy is amplification of a polymorphic tandem CAG repeat in the androgen receptor gene. "
2.	AndrogensIBA 02/01/1994 - "Aberrant androgen action and increased size of tandem CAG repeat in androgen receptor gene in X-linked recessive bulbospinal neuronopathy." 11/01/1994 - "Therefore, binding of androgen ([3H]dihydrotestosterone) to its specific receptor by genital skin fibroblasts cultured from a patient with X-linked recessive bulbospinal neuronopathy and confirmed androgen receptor mutation was studied. "
3.	Testosterone (Sustanon)FDA Link 02/01/1994 - "Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. "
4.	Dihydrotestosterone (Androstanolone)IBA 11/01/1994 - "Therefore, binding of androgen ([3H]dihydrotestosterone) to its specific receptor by genital skin fibroblasts cultured from a patient with X-linked recessive bulbospinal neuronopathy and confirmed androgen receptor mutation was studied. "
5.	Messenger RNA (mRNA)IBA 12/01/1994 - "Androgen receptor mRNA with increased size of tandem CAG repeat is widely expressed in the neural and nonneural tissues of X-linked recessive bulbospinal neuronopathy."
6.	Luteinizing Hormone (Lutropin)FDA LinkGeneric 02/01/1994 - "Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. "
7.	Hormones (Hormone)IBA 02/01/1994 - "Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. "
8.	Follicle Stimulating Hormone (Follitropin)FDA Link 02/01/1994 - "Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. "
9.	Fluoxymesterone (Halotestin)FDA Link 02/01/1994 - "Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. "
10.	EpitopesIBA 01/01/1990 - "Lower motor neurons of the spinal cord of patients with amyotrophic lateral sclerosis (ALS), Werdnig-Hoffmann's disease (WH), X-linked recessive bulbospinal neuronopathy (X-BSNP) and multiple system atrophy (MSA), all of which were known to involve the lower motor neurons, were immunohistochemically examined by using a monoclonal antibody (Ta-51) specific to phosphorylated epitopes of high molecular weight subunits of neurofilaments. "

Therapies and Procedures

1.	Therapeutics 05/01/2017 - "Kennedy disease (X-linked recessive bulbospinal neuronopathy): A comprehensive review from pathophysiology to therapy."