Anti-FAM111B Antibody Picoband®

SKU: A14246-2

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,IHC,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: FAM111B

Specificity:

Background: This gene encodes a protein with a trypsin-like cysteine/serine peptidase domain in the C-terminus. Mutations in this gene are associated with an autosomal dominant form of hereditary fibrosing poikiloderma (HFP). Affected individuals display mottled pigmentation, telangiectasia, epidermal atrophy, tendon contractures, and progressive pulmonary fibrosis. Alternative splicing results in multiple transcript variants encoding distinct isoforms. A paralog of this gene which also has a trypsin‐like peptidase domain, FAM111A, is located only 16 kb from this gene on human chromosome 11q12.1.

Immunogen: E.coli-derived human FAM111B recombinant protein (Position: Q328-D726). Human FAM111B shares 45.4% amino acid (aa) sequence identity with mouse FAM111B.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Khumalo, N. P., Pillay, K., Beighton, P., Wainwright, H., Walker, B., Saxe, N., Mayosi, B. M., Bateman, E. D. Poikiloderma, tendon contracture and pulmonary fibrosis: a new autosomal dominant syndrome? Brit. J. Derm. 155: 1057-1061, 2006. 2. Mercier, S., Kury, S., Shaboodien, G., Houniet, D. T., Khumalo, N. P., Bou-Hanna, C., Bodak, N., Cormier-Daire, V., David, A., Faivre, L., Figarella-Branger, D., Gherardi, R. K., and 18 others. Mutations in FAM111B cause hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis. Am. J. Hum. Genet. 93: 1100-1107, 2013.

Uniprot ID: Q6SJ93

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity:

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 85 kDa

Calculated Molecular Weight:

Gene ID: 374393

Protein Name: Serine protease FAM111B

Gene Full Name: FAM111 trypsin like peptidase B

Synonyms: CANP; FAM111B; Protein FAM111B