Rabbit Polyclonal MKS1(N-Term) Antibody

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     All lanes : Anti-MKS1 Antibody (N-Term) at 1:2000 dilution Lane 1: Human kidney lysate Lane 2: Human lung lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG,  (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 65 kDa Blocking/Dilution buffer: 5% NFDM/TBST.    

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     Immunofluorescent analysis of 4% paraformaldehyde-fixed, 0.1% Triton X-100 permeabilized HepG2 (human liver hepatocellular carcinoma cell line) cells labeling MKS1 with P34515 at 1/25 dilution, followed by Dylight® 488-conjugated goat anti-rabbit IgG  secondary antibody at 1/200 dilution (green). Immunofluorescence image showing cytoplasm staining on HepG2 cell line. Cytoplasmic actin is detected with Dylight® 554 Phalloidin  at 1/100 dilution (red). The nuclear counter stain is DAPI (blue).    

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     Overlay histogram showing HepG2 cells stained with P34515(green line).  The cells were fixed with 2% paraformaldehyde (10 min) and then permeabilized with 90% methanol for 10 min.  The cells were then icubated in 2% bovine serum albumin to block non-specific protein-protein interactions followed by the antibody (P34515,  1:25 dilution) for 60 min at 37ºC.  The secondary antibody used was Goat-Anti-Rabbit IgG, DyLight® 488 Conjugated Highly Cross-Adsorbed(OE188374) at 1/200 dilution for 40 min at 37ºC.  Isotype control antibody (blue line) was rabbit IgG1 (1μg/1x10^6 cells) used under the same conditions.  Acquisition of >10, 000 events was performed.    

The MKS1 (N-Term) antibody is a targeted reagent used to detect the N-terminal region of the MKS1 protein, a key component of the Meckel-Gruber syndrome (MKS) protein complex. MKS1 plays a critical role in ciliogenesis and the maintenance of primary cilia, which are essential for cellular signaling, tissue development, and homeostasis. Mutations in the MKS1 gene are linked to Meckel-Gruber syndrome, a severe autosomal recessive disorder characterized by renal cysts, central nervous system malformations, and polydactyly. The MKS1 protein interacts with other ciliary proteins (e.g., B9-domain proteins) to regulate ciliary assembly and function, particularly at the transition zone, a gatekeeping structure controlling cargo entry into cilia.

The MKS1 (N-Term) antibody is commonly utilized in research to study ciliary biology, developmental defects, and molecular mechanisms underlying ciliopathies. It is employed in techniques like Western blotting, immunofluorescence, and immunohistochemistry to visualize protein expression, localization, and dynamics in tissues or cell lines. By targeting the N-terminal domain, this antibody helps dissect structural or functional abnormalities in disease models, including those mimicking MKS or related disorders like Joubert syndrome. Its specificity for the N-terminal region ensures detection of full-length MKS1. aiding in distinguishing pathogenic truncations or mutations. Researchers also use this antibody to explore MKS1's role in signaling pathways, such as Hedgehog, and its interactions with other ciliopathy-associated proteins, advancing insights into therapeutic targets for ciliary diseases.