SURF-1 (F181) Peptide 
Datasheet
COA
MSDS
SHIP
Product Name :
SURF-1 (F181) Peptide Applications :
Blocking Background :
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. Alternative Name :
SURF 1; SURF-1; Surf1; SURF1; Surfeit 1; Surfeit locus protein 1; Swiss-Prot :
Q15526 Product :
1 mg/ml in DI water. Purification&Purity :
Synthetic peptide SURF-1 (F181). (Note: the amino acid sequence is proprietary). The purity is > 98%. Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
This peptide can be used with studies using BS2068 SURF-1 (F181) pAb.
SURF-1 (F181) Peptide Applications :
Blocking Background :
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. Alternative Name :
SURF 1; SURF-1; Surf1; SURF1; Surfeit 1; Surfeit locus protein 1; Swiss-Prot :
Q15526 Product :
1 mg/ml in DI water. Purification&Purity :
Synthetic peptide SURF-1 (F181). (Note: the amino acid sequence is proprietary). The purity is > 98%. Storage&Stability :
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity :
This peptide can be used with studies using BS2068 SURF-1 (F181) pAb.
Blocking peptide available as BS2068PP