84-2401-71　［取扱停止］SURF-1 (Surfeit locus Protein 1, SURF1, SURF-1) 100ul　226118

特徴

• 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.
• Applications:Suitable for use in Western Blot, Immunohistochemistry.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1:500-1:1000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.