Product Information
11358-1-PBS targets Harmonin in WB, IHC, IF/ICC, IP, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.
| Tested Reactivity | human, mouse, rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen |
CatNo: Ag1919 Product name: Recombinant human USH1C protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 1-392 aa of BC016057 Sequence: MDRKVAREFRHKVDFLIENDAEKDYLYDVLRMYHQTMDVAVLVGDLKLVINEPSRLPLFDAIRPLIPLKHQVEYDQLTPRRSRKLKEVRLDRLHPEGLGLSVRGGLEFGCGLFISHLIKGGQADSVGLQVGDEIVRINGYSISSCTHEEVINLIRTKKTVSIKVRHIGLIPVKSSPDEPLTWQYVDQFVSESGGVRGSLGSPGNRENKEKKVFISLVGSRGLGCSISSGPIQKPGIFISHVKPGSLSAEVGLEIGDQIVEVNGVDFSNLDHKEGRELFMTDRERLAEARQRELQRQELLMQKRLAMESNKILQEQQEMERQRRKEIAQKAAEENERYRKEMEQIVEEEEKFKKQWEEDWGSKEQLLLPKTITAEVHPVPLRKPKYDQGVEPE Predict reactive species |
| Full Name | Usher syndrome 1C (autosomal recessive, severe) |
| Calculated Molecular Weight | 552 aa, 62 kDa |
| Observed Molecular Weight | 68-73 kDa |
| GenBank Accession Number | BC016057 |
| Gene Symbol | USH1C/Harmonin |
| Gene ID (NCBI) | 10083 |
| RRID | AB_2214137 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Purification Method | Antigen affinity purification |
| UNIPROT ID | Q9Y6N9 |
| Storage Buffer | PBS only, pH 7.3. |
| Storage Conditions | Store at -80°C. |
Background Information
Harmonin, also known as USH1C, is a scaffold protein which interacts with other USH1 proteins (USH1B/myosin VIIa and USH1G/sans) to form complex and is implicated in proper development, morphogenesis, and structural integrity of the hair cell mechanoreceptor. Mutations of Harmonin cause Usher syndrome type 1 (congenital deafness and balance disorder, and early-onset sight loss).

















