DYNC1I1 Rabbit pAb
DYNC1I1 Rabbit pAb
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- 实验流程
- 背景知识
Application
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | Q9UI46 |
| Reactivity | Rat, Human |
| Predicted | Pig, Mouse, Rabbit, Chicken, Horse, Sheep |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 79283 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human DYNC1I1 |
| Epitope Specificity | 1-100/645 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Cytoplasm, cytoskeleton, cilium axoneme. |
| DISEASE | Defects in DNAI1 are the cause of primary ciliary dyskinesia type 1 (CILD1) [MIM:244400]. CILD1 is an autosomal recessive disorder characterized by axonemal 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 situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome. Defects in DNAI1 are the cause of Kartagener syndrome (KTGS) [MIM:244400]. KTGS is an autosomal recessive disorder characterized by the association of primary ciliary dyskinesia with situs inversus. Clinical features include recurrent respiratory infections, bronchiectasis, infertility, and lateral transposition of the viscera of the thorax and abdomen. The situs inversus is most often total, although it can be partial in some cases (isolated dextrocardia or isolated transposition of abdominal viscera). |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | The inner- and outer-arm dyneins, which bridge between the doublet microtubules in axonemes, are the force-generating proteins responsible for the sliding movement in axonemes. The intermediate and light chains, thought to form the base of the dynein arm, help mediate attachment and may also participate in regulating dynein activity. This gene encodes an intermediate chain dynein, belonging to the large family of motor proteins. Mutations in this gene result in abnormal ciliary ultrastructure and function associated with primary ciliary dyskinesia (PCD) and Kartagener syndrome. [provided by RefSeq, Jul 2008]. |
| Gene ID | 27019 |
|---|---|
| Other Names | Dynein axonemal intermediate chain 1, Axonemal dynein intermediate chain 1, DNAI1 |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=1 µg/Test |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | DNAI1 |
|---|---|
| Function | Component of dynein, a family of motor proteins essential for movement along microtubules (By similarity). Required for structural and functional integrity of cilia (By similarity). Part of the dynein complex of respiratory cilia. |
| Cellular Location | Dynein axonemal particle. Cytoplasm, cytoskeleton, cilium axoneme. Cell projection, cilium, flagellum. Cytoplasm, cytoskeleton {ECO:0000250|UniProtKB:Q8C0M8}. Note=Localizes to the manchette in elongating spermatids in a CFAP70-dependent manner {ECO:0000250|UniProtKB:Q8C0M8} |
| Tissue Location | Expressed in respiratory ciliated cells (at protein level). |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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