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Invitrogen™ SURF1 Polyclonal Antibody
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Catalog No. PIPA5110448
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100 μL
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PIPA5110448 100 μL
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Catalog No. PIPA5110448 Supplier Invitrogen™ Supplier No. PA5110448
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Description

Rabbit Polyclonal Antibody

Immunogen sequence: QVQRRKWKLN LIAELESRVL AEPVPLPADP MELKNLEYRP VKVRGCFDHS KELYMMPRTM VDPVREAREG GLISSSTQSG AYVVTPFHCT DLGVTILVNR GFVPRKKVNP ETRQKGQIEG EVDLIGMVRL TETRQPFVPE NNPERNHWHY RDLEAMARIT GAEPIFIDAN FQSTVPGGPI GGQTRVTLRN EHLQYI.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen SURF1
Applications ELISA, Western Blot
Classification Polyclonal
Concentration 2.15 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene SURF1
Gene Accession No. P09925, Q15526, Q9QXU2
Gene Alias 0610010F23Rik; Ab1-205; CMT4K; Surf1; Surf-1; surfeit 1; surfeit gene 1; surfeit locus protein 1
Gene Symbols SURF1
Host Species Rabbit
Immunogen Recombinant fusion protein containing a sequence corresponding to amino acids 80-275 of human SURF1 (NP_003163.1).
Purification Method Affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 20930, 64463, 6834
Target Species Human, Mouse, Rat
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG
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