XPA Human

Xeroderma Pigmentosum, Complementation Group A Human Recombinant
Cat. No.
BT1211
Source
Escherichia Coli.
Synonyms
XP1, XPAC, DNA repair protein complementing XP-A cells.
Appearance
Sterile Filtered clear solution.
Purity
Greater than 85.0% as determined by SDS-PAGE.
Usage
Prospec's products are furnished for LABORATORY RESEARCH USE ONLY. They may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

XPA Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 296 amino acids (1-273 a.a.) and having a molecular mass of 33.8 kDa.XPA is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
DNA repair protein complementing XP-A cells (XPA) is a member of the XPA family. This protein plays a crucial role in DNA excision repair by binding to damaged DNA sites. The binding affinity varies depending on the type of DNA damage (photoproduct) and the transcriptional activity of the affected DNA region. Defects in XPA protein function cause xeroderma pigmentosum complementation group A (XP-A), a rare autosomal recessive disorder. XP-A is characterized by hypersensitivity to sunlight, a high risk of developing skin cancers in sun-exposed areas, and potential neurological abnormalities.
Description
Recombinant human XPA protein is produced in E. coli. It is a single, non-glycosylated polypeptide chain consisting of 296 amino acids (with amino acids 1 to 273 present) and has a molecular weight of 33.8 kDa. The protein includes a 23 amino acid His-tag fused at the N-terminus to facilitate purification. It is purified using proprietary chromatographic techniques.
Physical Appearance
Clear, sterile solution after filtration.
Formulation
The XPA protein solution has a concentration of 1 mg/ml and is supplied in a buffer containing 20mM Tris-HCl (pH 8.0), 0.4M Urea, and 10% glycerol.
Stability
For short-term storage (up to 2-4 weeks), the protein should be stored at 4°C. For long-term storage, it is recommended to store the protein at -20°C. Adding a carrier protein (0.1% HSA or BSA) is recommended for extended storage durations. Avoid repeated freezing and thawing of the protein solution.
Purity
The purity of the XPA protein is greater than 85% as determined by SDS-PAGE analysis.
Synonyms
XP1, XPAC, DNA repair protein complementing XP-A cells.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSMAAADGA LPEAAALEQP AELPASVRAS IERKRQRALM LRQARLAARP YSATAAAATG GMANVKAAPK IIDTGGGFIL EEEEEEEQKI GKVVHQPGPV MEFDYVICEE CGKEFMDSYL MNHFDLPTCD NCRDADDKHK LITKTEAKQE YLLKDCDLEK REPPLKFIVK KNPHHSQWGD MKLYLKLQIV KRSLEVWGSQ EALEEAKEVR QENREKMKQK KFDKKVKELR RAVRSSVWKR ETIVHQHEYG PEENLEDDMY RKTCTMCGHE LTYEKM .

Product Science Overview

Introduction

Xeroderma Pigmentosum (XP) is a rare, autosomal recessive genetic disorder characterized by extreme sensitivity to ultraviolet (UV) rays from sunlight. This condition predominantly affects the skin and eyes, leading to a heightened risk of skin cancer and other complications. XP is divided into several complementation groups, with Xeroderma Pigmentosum, Complementation Group A (XPA) being one of the most studied due to its severe clinical manifestations.

Genetic Basis

The XPA gene, located on chromosome 9q22.33, encodes a protein crucial for the nucleotide excision repair (NER) pathway, a primary mechanism for repairing UV-induced DNA damage . Mutations in the XPA gene result in defective DNA repair, leading to the accumulation of DNA damage and subsequent cellular malfunction . Individuals with XPA mutations exhibit less than 2% of normal DNA repair activity .

Clinical Features

Patients with XPA typically present with severe photosensitivity, early onset of skin cancers (such as basal cell carcinoma, squamous cell carcinoma, and malignant melanoma), and various neurological abnormalities . Common symptoms include:

  • Skin: Early freckle-like lesions, poikiloderma, increased/decreased skin pigmentation, skin atrophy, telangiectasia, actinic keratoses, angiomas, and keratoacanthomas .
  • Eyes: Photophobia, conjunctivitis, keratitis, ectropion, and entropion .
  • Neurological: Mental deterioration, low intelligence, microcephaly, sensorineural deafness, hyporeflexia, spasticity, ataxia, and choreoathetosis .
Molecular Mechanism

The XPA protein plays a pivotal role in the NER pathway by recognizing and binding to damaged DNA sites. It interacts with other proteins to form a complex that unwinds the DNA, excises the damaged section, and replaces it with the correct DNA sequence . This process is essential for maintaining genomic stability and preventing mutations that could lead to cancer .

Human Recombinant XPA

Human recombinant XPA refers to the XPA protein produced through recombinant DNA technology. This involves inserting the XPA gene into a suitable expression system, such as bacteria or yeast, to produce the protein in large quantities. Recombinant XPA is used in research to study the NER pathway and develop potential therapeutic interventions for XP .

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