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Type
Conjugated Primary Antibody
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Conjugated with
ALEXA FLUOR® 594
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Host organism
Rabbit (Oryctolagus cuniculus)
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Target Protein Peptide
LMX1b/NPS1
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Specificity
This antibody reacts specifically with LMX1b/NPS1
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Modification
No modification has been applied to this antibody
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Modification site
None
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Clonality
Polyclonal Antibody
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Clone
Polyclonal Antibodies
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Concentration
1ug per 1ul
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Subcellular locations
N/A
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Antigen Source
KLH conjugated synthetic peptide derived from human LMX1b/NPS1
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Gene ID
N/A
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Swiss Prot
N/A
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Applications
IF(IHC-P)
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Applications with corresponding dilutions
IF(IHC-P)(1:50-200)
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Cross reactive species
Human (Homo sapiens), Mouse (Mus musculus), Rat (Rattus norvegicus)
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Cross Reactive Species details
No significant cross reactivity has been observed for this antibody for the tested species. However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species.
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Background information
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 method
Purified by Protein A.
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Storage
Water buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
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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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Also known as
LMX1b/NPS1 Polyclonal Antibody
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Other name
Anti-LMX1b/NPS1 Polyclonal
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Advisory
Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody, thus reducing its reactivity, specificity and sensitivity. For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube. Prior to use briefly centrifuge the vial to gather all the solution on the bottom.
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Properties
For facs or microscopy Alexa 1 conjugate.
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Conjugation
Alexa Fluor, ALEXA FLUOR® 594
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Conjugated
Alexa conjugate 1
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Description
This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided. Antibody for research use.
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Group
Polyclonals and antibodies
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About
Polyclonals can be used for Western blot, immunohistochemistry on frozen slices or parrafin fixed tissues. The advantage is that there are more epitopes available in a polyclonal antiserum to detect the proteins than in monoclonal sera.
