Rabbit Anti-LRRC6/FITC Conjugated antibody

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Leucine rich repeat containing 6; Leucine rich repeat containing protein 6; Leucine rich testis specific protein; Leucine-rich repeat-containing protein 6; Leucine-rich testis-specific protein; Lrrc6; LRTP; Protein TILB homolog; Testis spe

Cat:

SL13613R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human LRRC6

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

54kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
The leucine-rich repeat-containing protein 6 (LRRC6), also designated Leucine-rich testis-specific protein (LRTP), is a 466 amino acid protein that contains 3 LRR repeats and plays a role in spermatogenesis. The gene encoding LRRC6 maps to chromosome 8, which encodes approximately 800 genes. Translocation of portions of chromosome 8 with amplifications of the c-Myc gene are found in some leukemias and lymphomas, and typically associated with a poor prognosis. Chromosome 8 is also associated with Trisomy 8, Pfeiffer syndrome, congenital hypothyroidism and Waardenburg syndrome.

Function:
May play a role in dynein arm assembly, hence essential for proper axoneme building for cilia motility.

Subcellular Location:
Cytoplasm. Cell projection > cilium.

Tissue Specificity:
Expressed predominantly in testis and in nasal epithelial cells.

DISEASE:
Defects in LRRC6 are the cause of primary ciliary dyskinesia 19 (CILD19) [MIM:614935]. 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.

Similarity:
Belongs to the tilb protein family.
Contains 1 CS domain.
Contains 4 LRR (leucine-rich) repeats.
Contains 1 LRRCT domain.

Database links:

Entrez Gene: 23639 Human

Entrez Gene: 54562 Mouse

Entrez Gene: 299920 Rat

SwissProt: Q86X45 Human

SwissProt: O88978 Mouse

Unigene: 591865 Human

Unigene: 244890 Mouse

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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