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Rabbit Anti-KLHL7 Antibody (CSB-PA812891ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Kelch-like protein 7 Antibody,KLHL7 AntibodyMore alternative names for the antibody
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Kelch-like protein 7 protein (427-586AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Protein function|| |
Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex. The BCR(KLHL7) complex acts by mediating ubiquitination and subsequent degradation of substrate proteins. Probably mediates ‘Lys-48’-linked ubiquitination. .
|Protein tissue specificity|| |
Widely expressed, with highest levels in adult and fetal heart, CNS and adult testis. .
|Involvement in disease|| |
Cold-induced sweating syndrome 3 (CISS3) [MIM:617055]: A form of cold-induced sweating syndrome, an autosomal recessive disorder characterized by profuse sweating induced by cool surroundings (temperatures of 7 to 18 degrees Celsius). Patients manifest, in the neonatal period, orofacial weakness with impaired sucking and swallowing, resulting in poor feeding. Affected infants show a tendency to startle, with contractions of the facial muscles in response to tactile stimuli or during crying, trismus, abundant salivation, and opisthotonus. These features are referred to as Crisponi syndrome and can result in early death in infancy. Patients who survive into childhood have hyperhidrosis, mainly of the upper body, in response to cold temperatures, and sweat very little with heat. Additional abnormalities include a high-arched palate, nasal voice, depressed nasal bridge, inability to fully extend the elbows and kyphoscoliosis. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Retinitis pigmentosa 42 (RP42) [MIM:612943]: A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein cellular localization|| |
Nucleus . Cytoplasm . Note=Colocalizes with CUL3 in punctate structures at the perinuclear region of the cytoplasm. .
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|Product type|| |
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