Product Overview
Description
CLPP-00150762 is recombinant human HSPB1 protein
Protein Length
Full length protein
Nature
Recombinant Protein
Sequence
MTERRVPFSLLRGPSWDPFRDWYPHSRLFDQAFGLPRLPEEWSQWLGGSSWPGYVRPLPPAAIESPAVAAPAYSRALSRQLSSGVSEIRHTADRWRVSLDVNHFAPDELTVKTKDGVVEITGKHEERQDEHGYISRCFTRKYTLPPGVDPTQVSSSLSPEGTLTVEAPMPKLATQSNEITIPVTFESRAQLGGPEAAKSDETAAK
Sequence Similarities
Belongs to the small heat shock protein (HSP20) family.
Predicted Molecular Weight
27 kDa
Target Information
Alternative Names
Heat shock 27kDa protein; 28 kDa heat shock protein; CMT2F; DKFZp586P1322; epididymis secretory protein Li 102; Estrogen regulated 24 kDa protein; Estrogen-regulated 24 kDa protein; Heat shock 25kDa protein 1; Heat shock 27 kDa protein; Heat shock 27kD protein 1; Heat shock 27kDa protein 1; Heat shock 28kDa protein 1; Heat Shock Protein 27; Heat shock protein beta 1; Heat shock protein beta-1; heat shock protein family B (small) member 1; HEL-S-102; HMN2B; HS.76067; Hsp 25; HSP 27; Hsp 28; Hsp B1; Hsp25; HSP27; Hsp28; HspB1; HSPB1_HUMAN; SRP27; Stress responsive protein 27; Stress-responsive protein 27
Protein Function
Involved in stress resistance and actin organization.
Tissue Specificity
Detected in all tissues tested: skeletal muscle, heart, aorta, large intestine, small intestine, stomach, esophagus, bladder, adrenal gland, thyroid, pancreas, testis, adipose tissue, kidney, liver, spleen, cerebral cortex, blood serum and cerebrospinal fluid. Highest levels are found in the heart and in tissues composed of striated and smooth muscle.
Involvement in Disease
Defects in HSPB1 are the cause of Charcot-Marie-Tooth disease type 2F (CMT2F). CMT2F is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced. CMT2F onset is between 15 and 25 years with muscle weakness and atrophy usually beginning in feet and legs (peroneal distribution). Upper limb involvement occurs later. CMT2F inheritance is autosomal dominant.Defects in HSPB1 are a cause of distal hereditary motor neuronopathy type 2B (HMN2B). Distal hereditary motor neuronopathies constitute a heterogeneous group of neuromuscular disorders caused by selective impairment of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs.
Shipping & Handling
Constituents
0.32% Tris HCl, 2.61% Sodium chloride, 10% Glycerol (glycerin, glycerine), 0.08% DTT.
Shipping
Shipped at 4 °C.
Storage
Store at -20 °C for long term.
