AmyCo

Basic Information

Disease Name:

Amyotrophic Lateral Sclerosis 1

ISA Name:

No assigned nomenclature

Alternative Names:

ALS (Amyotrophic Lateral Sclerosis)
Amyotrophic Lateral Sclerosis With Dementia
Amyotrophic Lateral Sclerosis, Guam Form
Amyotrophic Lateral Sclerosis, Parkinsonism-Dementia Complex of Guam
Amyotrophic Lateral Sclerosis-Parkinsonism-Dementia Complex 1
Charcot Disease
Dementia With Amyotrophic Lateral Sclerosis
Gehrig's Disease
Guam Disease
Guam Form of Amyotrophic Lateral Sclerosis
Lou Gehrig Disease
Lou Gehrig's Disease
Lou-Gehrigs Disease
Motor Neuron Disease, Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis 1, Familial
FALS
Amyotrophic lateral sclerosis 1, Autosomal dominant

MeSH Description:

A degenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, fasciculation, hyperreflexia, dysarthria, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous astrocytes and atrophy of anterior spinal nerve roots and corticospinal tracts.

Type:

Amyloidosis

ICD-10 Classification

Diseases of the Nervous System

Tissue:

Central Nervous System (CNS)

Precursor Proteins:

Primary Name:	Superoxide dismutase [Cu-Zn]
Gene Name:	SOD1
Alternative Protein Names:	Superoxide dismutase [Cu-Zn] (EC 1.15.1.1) (Superoxide dismutase 1) (hSod1)
ISA Nomenclature:	No information available
Protein Length:	154
Precursor Protein Sequence:	MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ
UniProt AC:	P00441
UniProt ID:	SODC_HUMAN
AmyPro:	AP00084

Co-deposited Proteins:

No information available

MeSH:
ICD:
OMIM:
PubMed:

Protein Information