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Mouse Anti-DNAAF1 Recombinant Antibody (2C4) (CBMAB-L0313-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Dynein Axonemal Assembly Factor 1 (DNAAF1). The antibody can be used for immunoassay techniques, such as WB, FC.
See all DNAAF1 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat, Dog, Primate
Clone
2C4
Antibody Isotype
IgG1
Application
WB, FC

Basic Information

Immunogen
Full length human recombinant protein of human LRRC50(NP_848547) produced in HEK293T cell.
Specificity
Human, Mouse, Rat, Dog, Primate
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.4
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Dynein Axonemal Assembly Factor 1
Introduction
DNAAF1 is cilium-specific and is required for the stability of the ciliary architecture. DNAAF1 is involved in the regulation of actin-based brush border microvilli and microtubule-based cilia. Mutations in this gene are associated with primary ciliary dyskinesia-13. Alternative splicing leads to multiple transcript variants.
Entrez Gene ID
Human123872
Mouse68270
Rat361419
Dog479628
UniProt ID
HumanQ8NEP3
MouseQ9D2H9
RatQ6AYH9
DogF6URB8
Alternative Names
Dynein Axonemal Assembly Factor 1; Leucine-Rich Repeat-Containing Protein 50; LRRC50; Outer Row Dynein Assembly 7 Homolog (Chlamydomonas); Dynein; Axonemal; Assembly Factor 1; Dynein (Axonemal) Assembly Factor 1; Outer Row Dynein Assembly 7 Homolog
Function
Cilium-specific protein required for the stability of the ciliary architecture. Plays a role in cytoplasmic preassembly of dynein arms. Involved in regulation of microtubule-based cilia and actin-based brush border microvilli.
Biological Process
Axonemal dynein complex assembly Source: UniProtKB
Cilium assembly Source: UniProtKB
Cilium movement Source: BHF-UCL
Determination of digestive tract left/right asymmetry Source: BHF-UCL
Determination of liver left/right asymmetry Source: BHF-UCL
Determination of pancreatic left/right asymmetry Source: BHF-UCL
Epithelial cilium movement involved in determination of left/right asymmetry Source: BHF-UCL
Heart looping Source: BHF-UCL
Inner dynein arm assembly Source: BHF-UCL
Left/right pattern formation Source: BHF-UCL
Lung development Source: BHF-UCL
Motile cilium assembly Source: BHF-UCL
Outer dynein arm assembly Source: BHF-UCL
Regulation of cilium beat frequency Source: BHF-UCL
Cellular Location
Cytoplasm; Spindle pole; Cilium. In HEK293T cells, it is diffusely cytoplasmic and concentrates at the mitotic spindle poles, while in MDCK cells, it localizes in the cilium. In vivo, this protein is probably restricted to the cilium.
Involvement in disease
Ciliary dyskinesia, primary, 13 (CILD13):
A disorder characterized by abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome. At ultrastructural level, CILD13 is characterized by a marked reduction or absence of both dynein arms from the patients cilia.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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