LMX1b/NPS1 Antibody, ALEXA FLUOR 594

Catalog numberbs-11780R-A594
NameLMX1b/NPS1 Antibody, ALEXA FLUOR 594
Price€ 380.00
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Long nameLMX1b/NPS1 Polyclonal Antibody, ALEXA FLUOR 594 Conjugated
Also known asAnti-LMX1b/NPS1 PAb ALEXA FLUOR 594
CategoryConjugated Primary Antibodies
Conjugated withALEXA FLUOR® 594
Host OrganismRabbit (Oryctolagus cuniculus)
Target AntigenLMX1b/NPS1
SpecificityThis is a highly specific antibody against LMX1b/NPS1.
Modification SiteNone
ClonePolyclonal antibody
Concentration1ug per 1ul
SourceThis antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human LMX1b/NPS1
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 antigenNail-patella syndrome (NPS) is an autosomal dominant disorder characterized by dyplasia of finger nails, skeletal anomalies and, frequently, renal disease. NPS is caused by putative loss-of-function mutations in the transcription factor LMX1B. LMX1B belongs to the LIM-homeodomain family, members of which are known to be important for pattern formation during development. Twenty-two novel mutations may occur in the gene encoding LMX1B and the type and distribution of the mutations support the hypothesis that NPS is the result of haploinsufficiency for LMX1B. LMX1B is also necessary for normal development of the eye and in regulating dopaminergic neurogenesis and may be involved in developmental glaucoma and the aetiology of idiopathic Parkinson’s disease. Specifically, LMX1B along with LIM1 control the initial trajectory of motor axons in the developing mammalian limb. In addition, LMX1B directly regulates the coordinated expression of alpha 3(IV) and alpha 4(IV) collagen required for normal glomerular basement membrane (GBM) morphogenesis, and the dysregulation of LMX1B in GBM contributes to the renal pathology and nephrosis in NPS.
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 emission590nm/617nm
SynonymsLIM homeo box transcription factor 1 beta; LIM homeobox transcription factor 1 beta; LIM homeobox transcription factor 1-beta; LIM-homeobox protein 1.2; LIM/homeobox protein 1.2; LIM/homeobox protein LMX1B; LMX 1.2; LMX-1.2; LMX1.2; LMX1B; LMX1B_HUMAN; NPS 1; NPS1.
PropertiesFor facs or microscopy Alexa 1 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® 594
ConjugatedAlexa conjugate 1
French translationanticorps