SURF-1 (F181) pAb

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.

Prices direct from Bioworld Technology, Inc.

Quick response times

Exclusive Biosave savings/discounts

SPECIFICATIONS

Catalog Number

BS2068

Size

100ug/100ul

Applications

IHC, WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

liquid

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Antigen

Synthetic peptide, corresponding to amino acids 150-200 of Human SURF-1.

Species

SURF-1 (F181) pAb detects endogenous levels of SURF-1 protein.

Dilutions

WB: 1:500~1:1000\nIHC: 1:50~1:200

Swiss Prot

Q15526

Storage Temp

Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.

Concentration

1ug/ul