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Anti-TWIST1/2 Antibody Picoband® Fluoro647 Conjugated

Product Specifications

Background

Twist-related protein 1 (TWIST1) also known as class A basic helix–loop–helix protein 38 (bHLHa38) is a basic helix-loop-helix transcription factor that in humans is encoded by the TWIST1 gene. This gene encodes a basic helix-loop-helix (bHLH) transcription factor that plays an important role in embryonic development. The encoded protein forms both homodimers and heterodimers that bind to DNA E box sequences and regulate the transcription of genes involved in cranial suture closure during skull development. This protein may also regulate neural tube closure, limb development and brown fat metabolism. This gene is hypermethylated and overexpressed in multiple human cancers, and the encoded protein promotes tumor cell invasion and metastasis, as well as metastatic recurrence. Mutations in this gene cause Saethre-Chotzen syndrome in human patients, which is characterized by craniosynostosis, ptosis and hypertelorism.

Synonyms

ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial; CLPX

Gene Name

TWIST1/2

Gene ID

7291/117581

UniProt

Q15672/Q8WVJ9

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

A synthetic peptide corresponding to a sequence in the middle region of human TWIST1/2, identical to the related mouse sequences.

Clonality

Polyclonal

Tissue Specificity

Higher expression in skeletal muscle and heart and to a lesser extent in liver, brain, placenta, lung, kidney and pancreas.

Applications

Flow Cytometry

Field of Research

Cell Biology, Chaperones, Metabolism, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Pathways and Processes, Protein Trafficking, Proteolysis/Ubiquitin, Proteolytic Enzymes, Signal Transduction

Purification

Immunogen affinity purified.

Form

Liquid

Function

ATP-dependent specificity component of the Clp protease complex. Hydrolyzes ATP. Targets specific substrates for degradation by the Clp complex (PubMed:11923310, PubMed:22710082) . Can perform chaperone functions in the absence of CLPP. Enhances the DNA-binding activity of TFAM and is required for maintaining a normal mitochondrial nucleoid structure (PubMed:22841477) . ATP- dependent unfoldase that stimulates the incorporation of the pyridoxal phosphate cofactor into 5-aminolevulinate synthase, thereby activating 5-aminolevulinate (ALA) synthesis, the first step in heme biosynthesis. Important for efficient erythropoiesis through upregulation of heme biosynthesis (PubMed:25957689) .

References & Citations

1. Bialek, P., Kern, B., Yang, X., Schrock, M., Sosic, D., Hong, N., Wu, H., Yu, K., Ornitz, D. M., Olson, E. N., Justice, M. J., Karsenty, G. A Twist code determines the onset of osteoblast differentiation. Dev. Cell 6: 423-435, 2004. 2. Bourgeois, P., Bolcato-Bellemin, A.-L., Danse, J.-M., Bloch-Zupan, A., Yoshiba, K., Stoetzel, C., Perrin-Schmitt, F. The variable expressivity and incomplete penetrance of the twist-null heterozygous mouse phenotype resemble those of human Saethre-Chotzen syndrome. Hum. Molec. Genet. 7: 945-957, 1998. 3. Bourgeois, P., Stoetzel, C., Bolcato-Bellemin, A. L., Mattei, M. G., Perrin-Schmitt, F. The human H-twist gene is located at 7p21 and encodes a b-HLH protein that is 96% similar to its murine M-twist counterpart. Mammalian Genome 7: 915-917, 1996.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Applications Notes

6

Gene Name Synonym

Caseinolytic mitochondrial matrix peptidase chaperone subunit

Subcellular Location

Mitochondrion. Mitochondrion matrix, mitochondrion nucleoid.

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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