CABC1 Antibody, ALEXA FLUOR 555

Catalog numberbs-8071R-A555
NameCABC1 Antibody, ALEXA FLUOR 555
Price€ 380.00
Size100ul
  Get from shop
Long nameCABC1 Polyclonal Antibody, ALEXA FLUOR 555 Conjugated
Also known asAnti-CABC1 PAb ALEXA FLUOR 555
CategoryConjugated Primary Antibodies
Conjugated withALEXA FLUOR® 555
Host OrganismRabbit (Oryctolagus cuniculus)
Target AntigenCABC1
SpecificityThis is a highly specific antibody against CABC1.
ModificationUnmodified
Modification SiteNone
ClonalityPolyclonal
ClonePolyclonal antibody
Concentration1ug per 1ul
SourceThis antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human ADCK3/CABC1
Tested applicationsIF(IHC-P)
Recommended dilutionsIF(IHC-P)(1:50-200)
CrossreactivityHuman, Mouse, Rat
Cross-reactive species detailsDue to limited amount of testing and knowledge, not every possible cross-reactivity is known.
Background of the antigenMay be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.Tissue specificity:Ubiquitously expressed with a relatively greater abundance in heart and skeletal muscle.Involvement in disease:Defects in ADCK3 are a cause of coenzyme Q10 deficiency (CoQ10 deficiency). CoQ10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy.Defects in ADCK3 are the cause of spinocerebellar ataxia autosomal recessive type 9 (SCAR9) [MIM:612016]; also known as autosomal recessive cerebellar ataxia type 2 (ARCA2). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR9 is an autosomal recessive form characterized by gait ataxia and cerebellar atrophy with slow progression and few associated features. Patients can manifest brisk tendon reflexes and Hoffmann sign, mild psychomotor retardation, mild axonal degeneration of the sural nerve, exercise intolerance and elevated serum lactate.
PurificationPurified by Protein A.
Storage conditionsStore this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
Excitation emission553nm/568nm
Synonymsmitochondrial; aarF domain containing protein kinase 3; aarF domain-containing protein kinase 3; ADCK 3; ADCK3; ADCK3_HUMAN; CABC 1; Chaperone ABC1 activity of bc1 complex S.pombe like; Chaperone ABC1 activity of bc1 complex homolog; Chaperone ABC1 like; Chaperone activity of bc1 complex like; Chaperone activity of bc1 complex like mitochondrial; Chaperone activity of bc1 complex-like; Chaperone-ABC1-like; Coenzyme Q8 homolog; COQ 8; COQ8.
PropertiesFor facs or microscopy Alexa 1 conjugate.Very high photo stable ALEXA conjugate.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
ConjugationAlexa Fluor,ALEXA FLUOR 555
French translationanticorps