USH1C polyclonal antibody
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More Files
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Specification
Product Description
Goat polyclonal antibody raised against synthetic peptide of USH1C.
Immunogen
A synthetic peptide corresponding to human USH1C.
Sequence
DRKVAREFRHKVD-C
Host
Goat
Theoretical MW (kDa)
62.2, 101
Reactivity
Human
Specificity
This antibody is expected to recognize both reported isoforms (NP_005700.2 and NP_710142.1).
Form
Liquid
Purification
Antigen affinity purification
Concentration
0.5 mg/mL
Quality Control Testing
Antibody Reactive Against Synthetic Peptide.
Recommend Usage
ELISA (1:128000)
Western Blot (0.1-0.3 ug/mL)
Immunohistochemistry (10 ug/mL)
The optimal working dilution should be determined by the end user.Storage Buffer
In Tris saline, pH 7.3 (0.5% BSA, 0.02% sodium azide)
Storage Instruction
Store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Cell lysate)
USH1C polyclonal antibody (Cat # PAB6589) (0.1 ug/mL) staining of HEK293 lysate (35 ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
USH1C polyclonal antibody (Cat # PAB6589) (10 ug/mL) staining of paraffin embedded human kidney. Microwaved antigen retrieval with citrate buffer pH 6, HRP-staining. (Data have been obtained with EB06287 from a previous batch).Enzyme-linked Immunoabsorbent Assay
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Gene Info — USH1C
Entrez GeneID
10083Protein Accession#
NP_005700.2;NP_710142.1Gene Name
USH1C
Gene Alias
AIE-75, DFNB18, NY-CO-37, NY-CO-38, PDZ-45, PDZ-73, PDZ-73/NY-CO-38, PDZ73, ush1cpst
Gene Description
Usher syndrome 1C (autosomal recessive, severe)
Gene Ontology
HyperlinkGene Summary
This gene encodes a scaffold protein that functions in the assembly of Usher protein complexes. The protein contains PDZ domains, a coiled-coil region with a bipartite nuclear localization signal and a PEST degradation sequence. Defects in this gene are the cause of Usher syndrome type 1C and non-syndromic sensorineural deafness autosomal recessive type 18. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq
Other Designations
harmonin
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Interactome
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Disease
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Publication Reference
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A defect in harmonin, a PDZ domain-containing protein expressed in the inner ear sensory hair cells, underlies Usher syndrome type 1C.
Verpy E, Leibovici M, Zwaenepoel I, Liu XZ, Gal A, Salem N, Mansour A, Blanchard S, Kobayashi I, Keats BJ, Slim R, Petit C.
Nature Genetics 2000 Sep; 26(1):51.
Application:IF, Mouse, Mouse inner ear.
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A defect in harmonin, a PDZ domain-containing protein expressed in the inner ear sensory hair cells, underlies Usher syndrome type 1C.
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