LMX1b/NPS1 Antibody, ALEXA FLUOR 594
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Catalog number
bs-11780R-A594
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Price
Please ask
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Size
100ul
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Long name
LMX1b/NPS1 Polyclonal Antibody, ALEXA FLUOR 594 Conjugated
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Also known as
Anti-LMX1b/NPS1 PAb ALEXA FLUOR 594
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Category
Conjugated Primary Antibodies
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Conjugated with
ALEXA FLUOR® 594
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Host Organism
Rabbit (Oryctolagus cuniculus)
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Target Antigen
LMX1b/NPS1
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Specificity
This is a highly specific antibody against LMX1b/NPS1.
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Modification
Unmodified
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Modification Site
None
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Clonality
Polyclonal
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Clone
Polyclonal antibody
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Concentration
1ug per 1ul
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Source
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human LMX1b/NPS1
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Tested applications
IF(IHC-P)
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Recommended dilutions
IF(IHC-P)(1:50-200)
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Crossreactivity
Human, Mouse, Rat
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Cross reactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Background of the antigen
Nail-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.
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Purification
Purified by Protein A.
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Storage conditions
Store 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.
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Excitation emission
590nm/617nm
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Synonyms
LIM 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.
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Properties
For 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.
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Conjugation
Alexa Fluor, ALEXA FLUOR® 594
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Conjugated
Alexa conjugate 1
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French translation
anticorps
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Gene target
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Gene symbol
LMX1B
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Short name
Anti-LMX1b/NPS1
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Technique
Antibody, antibodies against human proteins, antibodies for
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Isotype
Immunoglobulin G (IgG)
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Label
ALEXA FLUOR 594
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Alternative name
LMX1b/NPS1 Polyclonal Antibody, ALEXA FLUOR 594
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Alternative technique
antibodies
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Gene info
MeSH Data
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Name
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Concept
Scope note:
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
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Tree numbers
- E05.196.401.143
- E05.301.300.096
- E05.478.566.320.200
- E05.601.262
- E05.601.470.320.200
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Qualifiers
ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data
Product images
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