TMEM67 encodes transmembrane protein 67, also known as Meckelin, a cilium-associated protein critical for ciliogenesis and maintenance. It localizes to the ciliary membrane and basal body, regulating vesicle trafficking and signal transduction through interactions with intraflagellar transport proteins.
Mutations in TMEM67 cause autosomal recessive Meckel-Gruber syndrome, characterized by renal cystic disease, central nervous system malformations, and hepatic fibrosis. It is linked to Hedgehog signaling pathway dysfunction, with no approved therapies targeting TMEM67 currently; research focuses on ciliopathy mechanisms and potential small molecule modulators.
TMEM67 Recombinant Monoclonal Antibody (CSB-RA780441A0HU)
Validated Data
Western Blot
Positive WB detected in: K562 whole cell lysate(30µg), A431 whole cell lysate(30µg), Hela whole cell lysate(30µg), U-251MG whole cell lysate(30µg), HEK293 whole cell lysate(30µg), MCF-7 whole cell lysate(30µg), Rat brain tissue lysate(30µg)
All lanes: Meckelin polymerase beta antibody at 1:1000
Secondary
Goat polyclonal to rabbit IgG at 1/40000 dilution
Predicted band size: 112 kDa
Observed band size: 112, 122 kDa
Exposure time:1min
Immunofluorescence staining of U-251MG cell with CSB-RA780441A0HU at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Overlay Peak curve showing 786-O cells stained with CSB-RA780441A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
TMEM67 Antibody (CSB-PA689243LA01HU)
Validated Data
Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA689243LA01HU at dilution of 1:100
Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA689243LA01HU at dilution of 1:100
Immunofluorescent analysis of HepG2 cells using CSB-PA689243LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
The following TMEM67 reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
TMEM67 Antibodies for Homo sapiens (Human)
| Code | Product Name | Species Reactivity | Application |
|---|---|---|---|
| CSB-PA023871GA01HU | TMEM67 Antibody | Human | ELISA,IF |
| CSB-PA689243LA01HU | TMEM67 Antibody | Human | ELISA, IHC, IF |
| CSB-PA689243LB01HU | TMEM67 Antibody, HRP conjugated | Human | ELISA |
| CSB-PA689243LC01HU | TMEM67 Antibody, FITC conjugated | Human | |
| CSB-PA689243LD01HU | TMEM67 Antibody, Biotin conjugated | Human | ELISA |
| CSB-RA780441A0HU | TMEM67 Recombinant Monoclonal Antibody | Human, Rat | ELISA, WB, IF, FC |
TMEM67 Proteins for Homo sapiens (Human)
| Code | Product Name | Source |
|---|---|---|
| CSB-YP689243HU CSB-EP689243HU CSB-BP689243HU CSB-MP689243HU CSB-EP689243HU-B |
Recombinant Human Meckelin (TMEM67), partial | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |