Invitrogen™ SURF1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA536198

Catalog No. PIPA536198

Description

This antibody detects endogenous protein at a molecular weight of 33 kDa. Purity is >95% by SDS-PAGE.

The SURF-1 protein demonstrates a vital role in the assembly of complex IV (CIV or COX) of the mitochondrial respiratory chain. Expressed in the inner mitochondrial membrane, mutations of the SURF-1 gene generally cause cytochrome c oxidase complex IV deficiency. Shortage of complex IV leads to Leigh syndrome, a severe neurological disorder. Leigh syndrome patients are usually subject to rapidly progressive encephalopathy, characterized by necrotic lesions in subcortical brain regions. SURF-1 mutations correlate to high post-implantation embryonic lethality as well as early-onset mortality of post-natal individuals. Considerable deficit in muscle strength and motor performance is also a profound and isolated defect of SURF-1 activity in skeletal muscle and liver. Heart, brain and skeletal muscle morphological abnormalities frequently occur due to SURF-1 mutations.

Specifications

• Antigen: SURF1
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: SURF1
• Gene Alias: 0610010F23Rik; Ab1-205; CMT4K; Surf1; Surf-1; surfeit 1; surfeit gene 1; surfeit locus protein 1
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 20930, 64463, 6834
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: P09925, Q15526, Q9QXU2
• Gene Symbols: SURF1
• Immunogen: Synthetic peptide corresponding to amino acids 150-200 of human SURF-1.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG