ATPAF2 rabbit pAb

ATPAF2 rabbit pAb

AO-06-ES1731-50

ATPAF2 rabbit pAb 50μL

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Antibody Product Overview

ELK.NoES1731
Product nameATPAF2 rabbit pAb
ReactivityHuman;Mouse
ApplicationsWB;ELISA
Other nameATPAF2; ATP12; LP3663; ATP synthase mitochondrial F1 complex assembly factor 2; ATP12 homolog
Size50μL
Unit price ($)148
Human gene ID91647
Human Swiss-ProtQ8N5M1
SourceRabbit
IsotypeIgG
TargetATPAF2
Fields
Gene nameATPAF2
Protein nameATP synthase mitochondrial F1 complex assembly factor 2
Human gene linkView Human Gene
Human Swiss linkView Human Swiss-Prot
Mouse gene ID246782
Mouse gene linkView Mouse Gene
Mouse Swiss-ProtQ91YY4
Mouse Swiss linkView Mouse Swiss-Prot
Rat gene ID
Rat gene link
Rat Swiss-Prot
Rat Swiss link
ImmunogenThe antiserum was produced against synthesized peptide derived from human ATPAF2. AA range:21-70
SpecificityATPAF2 Polyclonal Antibody detects endogenous levels of ATPAF2 protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
ClonalityPolyclonal
DilutionWestern Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration1 mg/ml
Storage stability-20°C/1 year
Molecular Weight (Da)
Observed band (KD)35kD
BackgroundATP synthase mitochondrial F1 complex assembly factor 2(ATPAF2) Homo sapiens This gene encodes an assembly factor for the F(1) component of the mitochondrial ATP synthase. This protein binds specifically to the F1 alpha subunit and is thought to prevent this subunit from forming nonproductive homooligomers during enzyme assembly. This gene is located within the Smith-Magenis syndrome region on chromosome 17. An alternatively spliced transcript variant has been described, but its biological validity has not been determined. [provided by RefSeq, Jul 2008],
Functiondisease:Defects in ATPAF2 are the cause of complex V mitochondrial respiratory chain ATPAF2 subunit deficiency (ATPAF2 deficiency) [MIM:604273]; also called ATP synthase deficiency or ATPase deficiency. ATPAF2 deficiency seems to be an early presenting disease in which lactic acidosis, dysmorphic features, and methyl glutaconic aciduria can be major clues in the diagnosis. Dysmorphic features include a large mouth, prominent nasal bridge, micrognathia, rocker-bottom feet and flexion contractures of the limbs associated with camptodactyly. Patients are hypertonic and have an enlarged liver, hypoplastic kidneys and elevated lactate levels in urine, plasma and cerebro spinal fluid (CSF).,function:May play a role in the assembly of the F1 component of the mitochondrial ATP synthase (ATPase).,similarity:Belongs to the ATP12 family.,subunit:Interacts with ATP5A1.,tissue specificity:Widely expr
Subcellular locationMitochondrion .
ExpressionWidely expressed.

Additional Images

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Western Blot analysis of various cells using ATPAF2 Polyclonal Antibody
Image 2
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Western blot analysis of lysates from Jurkat cells, using ATPAF2 Antibody. The lane on the right is blocked with the synthesized peptide.
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: AO-06-ES1731-50
: 10 Produits
Hurry! only 10 items left in stock.

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