货号:ABE57
品牌:Merck Millipore
规格:
目录价:¥3639.00
市场价格:¥3093.15
会员价格:¥2911.20
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Description:
Anti-LMX-1.2 Antibody | ABE57
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Promotional Text:
Special Shipping Offer on Antibodies100% Performance Guaranteed
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Molecular Weight:
~ 42 kDa observed
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Immunogen:
KLH-conjugated linear peptide corresponding to human LMX-1.2.
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Isotype:
IgG
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Background Information:
LIM/homeobox protein 1.2 (LMX-1.2) belongs to the LIM homeodomain protein family and is comprised of 2 LIM zinc-binding domains and a single homeobox DNA-binding domain. LMX-1.2 is a required for determination of dorsal limb fate at the autopodal and zeugopodal levels during development, and is necessary for the normal development of eye, kidney, and skull. Expression is reported in the majority of tissues, with highest observed levels found in duodenum, pancreatic islets, skeletal muscle, testis, and thyroid. It is observed in the CNS, specifically is the isthmus, developing dopenergic and serotonergic neurons within the midgrain and hindbrain, roof and floor plate of the neural tube, and in a small group of dorsal spinal chord interneurons. Defects in expression causes nail-patella syndrome (NPS) which results in renal dysplasia and aberrant skeletal patterning.
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Species Reactivity:
Human
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Species Reactivity Note:
Demonstrated to react with human.
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Application Notes:
Immunohistochemistry Analysis: A representative lot was used by an independent laboratory in IH. (Dreyer, S. D., et al. (2000). Human Molecular Genetics. 9(7):1067-1074).
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Control:
Human fetal skeletal muscle tissue lysate
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Quality Assurance:
Evaluated by Western Blot in human fetal skeletal muscle tissue lysate.Western Blot Analysis: 1:1,000 dilution of this antibody detected LMX-1.2 on 10 µg of human fetal skeletal muscle tissue lysate.
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Purification Method:
Unpurified
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Presentation:
Rabbit polyclonal serum containing 0.5% sodium azide.
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Storage Conditions:
Stable for 1 year at -20°C from date of receipt.Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.
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UniProt Number:
O60663
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Entrez Gene Number:
NP_002307
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Gene Symbol:
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LMX1B
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LMX1.2
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LMX-1.2
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NPS1
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Alternate Names:
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LIM homeobox transcription factor 1, beta1
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LIM/homeobox protein LMX1B
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LIM/homeobox protein 1.2
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LIM homeobox transcription factor 1-beta
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Usage Statement:
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Key Applications:
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Western Blotting
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Immunohistochemistry
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Entrez Gene Summary:
This gene encodes a member of LIM-homeodomain family of proteins containing two N-terminal zinc-binding LIM domains, 1 homeodomain, and a C-terminal glutamine-rich domain. It functions as a transcription factor, and is essential for the normal development of dorsal limb structures, the glomerular basement membrane, the anterior segment of the eye, and dopaminergic and serotonergic neurons. Mutations in this gene are associated with nail-patella syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq].
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UniProt Summary:
FUNCTION: Essential for the specification of dorsal limb fate at both the zeugopodal and autopodal levels.SUBCELLULAR LOCATION: Nucleus Potential. TISSUE SPECIFICTY: Expressed in most tissues. Highest levels in testis, thyroid, duodenum, skeletal muscle, and pancreatic islets.INVOLVEMENT IN DISEASE: Defects in LMX1B are the cause of nail-patella syndrome (NPS) [MIM:161200]; also known as onychoosteodysplasia. NPS is a disease that cause abnormal skeletal patterning and renal dysplasia. SEQUENCE SIMILARITIES: Contains 1 homeobox DNA-binding domain.Contains 2 LIM zinc-binding domains.SEQUENCE CAUTION: The sequence CAH70294.1 differs from that shown. Reason: Erroneous initiation. The sequence CAI40917.1 differs from that shown. Reason: Erroneous initiation.
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Product Name:
Anti-LMX-1.2
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Antibody Type:
Polyclonal Antibody
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Qty/Pk:
100 µL
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Format:
Serum
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Host:
Rabbit
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