Renal dysplasia is an unusual clinical consequence of the kidney, and its typical histopathological manifestations are adnexa starting from glomeruli and renal tubules, chondroid metaplasia and so on. In recent years, through the application of molecular technology, the target gene and in-situ cloning have molecular regularity. The study on the development mechanism of normal mammals' kidneys has a better understanding of the pathogenesis of congenital renal dysplasia. In this paper, the molecular biology research status of reproductive renal dysplasia is discussed, and the good relationship between several gene mutations, replication regulation obstacles and expression changes, including growth factors, and kidney diseases is discussed. Renal dysplasia is a congenital disease caused by the failure of normal growth and development of the kidney. In the past, little was known about its mechanism. With the development and application of molecular biology technology, we have a deeper understanding of the mechanism of renal dysplasia from the molecular biology level. This paper introduces the research progress of this problem in the short term. 1 Before nephrogenesis and renal dysplasia, the kidneys of normal mammals were located between mesoderms, which differentiated to form renal ducts. After being further induced to the ureter buds to form renal ducts, the fresh renin on both sides of the final embryo was divided into renal embryoids under the guidance of the ureter buds. The development of renal embryos was completed by the ureter buds and the post-renal embryoids. The former grew into renal pelvis, renal calyces and collecting ducts in stages, while the latter grew into renal tubules and glomeruli, and finally renal tubules and glomeruli. If the ureter bud and the hindkidney embryo base can't grow and be absorbed according to the normal degree. Mely caused renal dysplasia. Renal dysplasia can be partial or complete. Most types of renal dysplasia are accompanied by cysts, and various forms that promote renal development have a common mechanism. Clinical common congenital renal dysplasia includes polycystic kidney disease, obstructive renal dysplasia and gene-related renal dysplasia. The important features of histopathology are primary active renal tubules and metaplastic structures. Complete list of renal dysplasia on one side, which can be asymptomatic. In most cases of hypoplasia, Shuang Ye of kidney promotes gene mutation to play an important role in normal kidney development. Unilateral disease may be caused by an acquired injury, which destroys the normal expression of genes, and then affects the production of protein with mature kidney. 2 Common types of renal dysplasia 2. 1 Congenital polycystic renal dysplasia Polycystic renal dysplasia (PCO) is a common complete renal dysplasia, mostly unilateral lesions (14-20% is bilateral), the affected kidney loses its normal shape, is replaced by cysts of irregular size, and has lost renal function and is often accompanied by ureteral obstruction, which is the abdomen of newborns. Polycystic dysplasia kidney has a kidney-shaped structure, and most cases are accompanied by a closed ureter. Polycystic kidney in early pregnancy is of great significance, including normal development. Urinary loss is introduced through the embryonic island of kidney and branch ducts. At this stage, cystic changes can be identified in all segments of nephron [1]. Histopathological changes of polycystic kidney include cystic changes of primitive renal tubules, disordered expansion and structure, obvious peritubular reactivity, formation of fibromuscular chains, and tissue transformation marked by cartilage components. 2.2 Congenital obstruction Renal dysplasia Congenital urinary tract obstruction occupies the ureter and bladder, and congenital posterior urethral valve is an important cause of urinary system obstruction in infants. The histological features of congenital obstructive kidney are similar to those of polycystic children, including nephron segments transforming into renal capsule, expanding in nature and disordered in structure, obvious hypoplasia of bone marrow and straight small vessels, fibromuscular chains around the tubes, various forms of glomeruli and growing nephron segments. Like polycystic kidney dysplasia, congenital obstructive kidney shows a series of diseases, and its degree is related to the time when the increase of urine flow resistance occurs during embroidery [2]. Chromosome inheritance with renal dysplasia syndrome is autosomal dominant with m-tip and finger (foot) malformation (Apert' s) Chester Gallery, nutritional asphyxia, obesity, low reproductive function and other autosomal recessive autosomal dominant camping growth abnormalities. Autosomal brain-liver-kidney (Passarge' s) autosomal susceptibility Fryns's autosomal susceptibility Goemine' s X-. Hall-pars's Autosomal Proximity of Send Mark Madden-Walker's Autosomal Proximity of McCott-gruber's Autosomal Proximity of Miranda's Autosomal Proximity of Senro-Loken? Trisomy 16- 18 (Edwards) trisomy 13- 15 (Patau) trisomy 2 1 (Down) autosomal dominant Von Hippel-Lindau autosomal dominant- E renal hypoplasia syndrome is a genetic indication group including renal capsule hypoplasia and other abnormalities (see the table below). Now, we will clarify some syndromes, their special genes and protein's defects. The incidence of dysplasia phenotype is now a band, which eventually promotes the phenotype of children affected by other genes. Dysplasia generally contains a variety of organs, indicating that defective genes are related to the basis of normal organ development. Histopathological findings showed that the patients with this syndrome may have large capsular formation (such as tuberous sclerosis), and the patients with severe syndrome may have abnormal capsular growth and renal failure (Meckel-gruber syndrome). 3 Molecular Biology of Renal Dysplasia At present, it is found that there are many genes related to renal dysplasia, such as wt- 1, Pax-2, GDNF, f-2, BMP-7, PDGF, Wnt-4 and so on, which are expressed after embryo. Pax-2, c-ret, BMP-7, α3β 1 were expressed in ureteral buds. When these genes are missing or destroyed, the kidney cannot grow normally [3]. Sonnenberg et al. [4] used specific antibodies and emission markers to guide the study of complement RNA and DNA probes, and determined the specific expression positions of polypeptide growth factor, heparin structural growth factor and its receptor, extracellular matrix members and cell surface total aggregation genes in renal development. For example, hepatocyte growth factor is mainly expressed after renal embryo, while its receptor c-met is expressed in the epidermis of ureteral embryo. This peptide and its receptor are exposed in two types of thin upper ureter, and the formation of ureteral catheter is introduced to the target of renal arch. Schuchardt and soon [5] found some genes and polypeptides that affected the growth of kidney by using gene recombination and preparing homozygous null mutation mice, such as transfer growth factor-β, hepatocyte growth factor and insulin-like growth factor-II. According to the final saw display, it was inferred that certain genes played a role in normal kidney. Tyrosine-activated proteasome receptor c-ret is a neurotrophic factor expressed in branched ureteral catheter and ligand-glial cells. When the c-ret gene of mice is destroyed, the whole kidney is stunted. Protein encoded by replication factor gene can bind to DNA and has the function of regulating the expression of other genes. Wilms' tumorgene WT- 1 and Pax2 encode replication factors in mammalian kidney growth, and their expression forms affect the differentiation of renal cells [6,7]. Gene syndrome is related to abnormal renal shape. Some of the diseases listed in the table are hereditary, and some have located specific gene defects by in-situ cloning technology [8]. Family members with these syndromes will have significant phenotypic variation. This situation is invalid in homozygotes. The changes seen in the changed mice are similar, that is, the final phenotype of the kidney is determined by the genetic background of the experimental mice. There are serious species differences in kidney development. Nes defect, or the final result of many gene regulation obstacles during embryonic development. The natural epidermis between the kidneys. The transformation process, branching and growth of mis are guided by a complex and huge gene system, some genes are kidney-specific and some are non-specific. Although some growth factor genes are sometimes actively expressed in the kidney, when they are destroyed, they do not necessarily block the normal growth of the kidney, which means that the genes normally expressed in the growing kidney overlap in function [9]. Another possibility is that the destruction of this normal expression form occurred in a certain period of renal dysplasia, or it was caused by renal dysplasia. The latter renal fire can lead to renal dysplasia. In addition, the gene disease should be expressed in a misplaced way, which may play a role in renal dysplasia. Clinically, there are two parallel cases of isolated polycystic kidney dysplasia and obstructive renal dysplasia. Congenital and experimental single gene mutations can lead to abnormal development of cystic kidney, and the mutations of these genes can change each other. Theoretically, the mutation can affect: (1) embryonic development and the expression of non-peptides and matrix proteins in the ureter; (2) The ability of ureter drive tube to respond to the signal of renal embryo posterior base; (3) After the start of ureteral catheter expression, it lost the ability to maintain the protein needed for the introduction of renal embryo basal epidermis; (4) The ability of the hindkidney embryo to respond to these alphanumeric characters; (5) The ability of cells to respond to the signals of ureter bud and posterior renal embryo base [10]. Recently, glucose phosphate adenosine triphosphate glycoprotein gene, called GPC3 gene, has been isolated. GPC3 deficiency is related to polycystic kidney dysplasia [1 1]. Although single-gene and multi-gene defects can eventually lead to renal dysplasia, their phenotypes can first determine the imbalance of gene regulation or expression changes, such as congenital obstruction and pouch renal dysplasia [12, 13]. The kidney of polycystic animals is poorly developed, and there are natural growth factor genes in the changes of cyst epidermis. Angiotensin and transfer growth factor were overexpressed in the obstructive growing kidney of mice [14]. The investigation shows that Pax2 and Bcl-2, which promote the changes of bladder in rubber tube epithelium, are also over-expressed after the abnormal development of kidney [15, 16]. This study may provide important clues to the pathogenesis of various forms of renal dysplasia.

Study on congenital renal dysplasia and its molecular biology

Renal hypoplasia is the clinical consequence of renal abnormality, and its typical histopathological feature is metaplasia.

Primary glomeruli and renal tubules, chondroid metaplasia, etc. In recent years, through the application of molecular technology such as target gene and in-situ cloning.

The molecular regulation mechanism of renal development in normal mammals was studied. The pathogenesis of congenital renal dysplasia is as follows

Learn more. In this paper, the molecular biology research of congenital renal dysplasia in recent years will be discussed, and its development prospect will be prospected

Relationship between several gene mutations, transcription regulation disorders and expression changes including growth factors and renal dysplasia

Discuss it.

Renal dysplasia is a congenital disease caused by the abnormal growth and development of the kidney, which has been caused in the past.

Little is known about its mechanism. With the development and application of molecular biotechnology, the occurrence of kidney is expounded from the molecular mechanism.

We have a deeper understanding of the occurrence of renal dysplasia from the molecular biology level. This paper expounds the recent problems.

The research progress of this paper is introduced.

1 nephrogenesis and renal dysplasia

The kidney of normal mammals is located in mesoderm, which differentiates to form the anterior renal duct.

It leads to the formation of mesonephric duct to ureter bud, and renin on both sides of embryo end is induced to differentiate into post-renin.

Kidney embryo base, the embryonic development of kidney is completed by ureter bud and posterior kidney embryo base, the former develops step by step.

It enters the renal pelvis, calyx and collecting duct, and the latter develops into renal tubules and glomeruli, and finally the renal tubules are connected with collecting duct.

Constitute a normal nephron. If the ureter bud and the posterior renal embryo base can not develop and arrange according to the normal degree

Then it will cause renal dysplasia. Renal dysplasia can be partial or complete. Most types

Renal dysplasia with cyst accounts for%, suggesting that all forms of dysplasia have the same formation mechanism.

Clinical common congenital renal dysplasia includes polycystic kidney disease, obstructive renal dysplasia and their relationship with genes.

Related renal dysplasia. An important feature of histopathology is the appearance of primitive renal tubules and metaplastic cartilage. Integrity table

Lateral renal dysplasia can be asymptomatic. In most cases of dysplasia, renal defects are bilateral, which shows that

Gene mutation plays an important role in normal kidney development. Unilateral diseases may be caused by acquired injuries,

This damage destroys the normal expression of genes, and then affects the production of protein, and protein is very important for kidney maturation.

Two Common Renal Dysplasia

2. 1 congenital polycystic kidney dysplasia

Multiple cystic renal dysplasia is a common integrity.

Renal dysplasia is mostly unilateral (14-20% is bilateral), and the affected kidney loses its normal shape and is irregular.

Cysts, renal insufficiency and ureteral obstruction are the most common abdominal masses in newborns.

One of the reasons.

Polycystic dysplasia has a kidney-shaped structure outside the kidney, and most cases are accompanied by ureteral closure. be pregnant

Early polycystic kidney contains essential components for normal development, including uninduced posterior renal embryonic island and branched urine infusion.

At this stage, cystic changes can be identified in all segments of nephron [1]. Postnatal polycystic kidney disease

Histopathological changes include cystic degeneration, enlargement and structural destruction of primitive renal tubules, as well as obvious peritubular characteristics.

The matrix of reaction, the formation of fibromuscular ring, tissue transformation marked by cartilage components, etc.

2.2 Congenital obstructive renal dysplasia

Congenital urinary tract obstruction often occurs at the junction of ureter and bladder in anatomical position.

Urethral valve is an important cause of urinary system obstruction in infants. Histological characteristics of congenital obstructive kidney and polycystic kidney disease.

Renal dysplasia is similar, including cystic changes in nephron segments such as glomerulus, interstitial swelling and structural damage, and bone marrow

There are obvious parenchymal and small straight vascular hypoplasia, fibrous muscle ring around the catheter, various forms of glomeruli and developing kidneys.

Unit paragraph. Like polycystic kidney dysplasia, congenital obstructive kidney shows a series of diseases, the degree of which is related to

The occurrence time of urine flow obstruction in embryo is related [2].

Renal dysplasia syndrome

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Chromosome genetic form of syndrome

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Autosomal dominant inheritance of pointed syndactyly

Asphyxia caused by autosomal recessive thoracic dystrophy

Autosomal recessive inheritance, such as obesity and reproductive dysfunction.

Gill-ear-kidney autosomal dominance

Autosomal recessive inheritance of Campomelic dysplasia

Brain-liver-kidney autosomal recessive inheritance

Fringe autosomal recessive inheritance

Goemine is x-connected

Goldston (hereditary telangiectasia) autosomal recessive?

From Hall pars's

Autosomal recessive inheritance of Ifemark

Recessive inheritance of Madden-Walker autosome

Recessive inheritance of Mei-Gru autosome

Miranda autosomal recessive inheritance

Autosomal recessive inheritance of Senlor-Loken?

Trisomy 16- 18 (Edwards)

Trisomy 13- 15 (Pato)

Trisomy 2 1 (downward)

Autosomal dominance of tuberous sclerosis

Von hippel-Lindau autosomal dominance

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2.3 Renal hypoplasia syndrome

Renal dysplasia syndrome is a hereditary syndrome, including renal malformation, such as cystic dysplasia (see table

)。 Now, some syndrome-specific genes and protein's defects have been clarified. The penetrance of dysplasia phenotype is

Now, a band shows that there are other genes that affect the final phenotype of the kidney. Dysplasia usually involves multiple organs,

It shows that defective genes are related to the basis of normal organogenesis. Pathohistology found that mild cases of this syndrome may be.

Large cysts (such as tuberous sclerosis) appear, and in severe cases, cystic dysplasia and renal failure may occur (Meckel-

Gruber syndrome)

Molecular Biology of Renal Dysplasia

At present, many genes are found to be related to renal dysplasia, such as WT- 1, Pax-2, GDNF and B.

F-2, BMP-7, PDGF, Wnt-4 and other genes were expressed in the embryonic base of the posterior kidney. Pax-2、c-ret、BMP-7、α3β

1 was expressed in ureteral bud. When these genes are lacking or destroyed, the kidney cannot develop normally [3]

]。 Sonnenberg et al. [4] studied specific antibodies and radiolabeled complement RNA and DNA probes to determine

Polypeptide growth factor, heparin structural growth factor and its receptor, extracellular matrix molecules and cell surface modification were introduced.

Specific expression positions of synaptophysin and other genes in renal development. For example, hepatocyte growth factor is mainly in the post-nephroblastoma gene.

Expression, and its receptor c-met is expressed in ureteral reproductive epithelium. There are two types of this polypeptide and its receptor.

Intracellular expression showed that ureteral catheter could induce the formation of posterior renal interstitium. Schuchardt et al [5] passed.

Through gene recombination and preparation of homozygous zero mutant mice, some genes and polypeptides affecting kidney development were found, such as

Transfer growth factor-β, hepatocyte growth factor, insulin-like growth factor-Ⅱ, according to the final table.

Type A infers the role of specific genes in normal nephrogenesis. Tyrosine kinase receptor c-ret in branched ureteral catheter

Tube and ligand-glial-derived neurotrophic factor. When the mouse c-ret gene is destroyed, it is composed of

Resulting in hypoplasia of the whole kidney. Protein encoded by transcription factor gene can bind to DNA and has the function of regulating other gene tables.

The function of. During the development of mammalian kidney, Wilms' tumor genes WT- 1 and Pax2 both encode transcription factors.

Its expression forms affect the differentiation of renal cells [6, 7]. Genetic syndrome is related to abnormal kidney formation, as listed in the table.

Some diseases and syndromes are inherited, and some specific gene defects have been located by in-situ cloning technology [

8]。 These syndromes may have significant phenotypic variation in family members. This situation is not true in homozygotes.

Mutant mice see similar variation, that is, the final phenotype of kidney depends on the genetic background of experimental mice.

The occurrence of renal dysplasia is caused by several different genetic defects or teratogenic factors encountered during embryonic development.

The final result of various genetic regulatory obstacles. Renal Interstitial-Epithelial Transformation and the Bifurcation and Growth of Ureter

, is guided by a complex and huge gene system, some genes are kidney-specific and some are non-specific.

Yes Some growth factor genes, although they are active in nephrogenesis, will not produce shadows when they are destroyed.

It affects the normal development of the kidney, which means that the genes normally expressed in the developing kidney overlap in function [9]. another

One possibility is that the destruction of this normal expression plays a role in the occurrence and development of renal dysplasia, or

Is the cause of renal dysplasia.

Renal interstitial defects can lead to renal dysplasia. In addition, gene imbalance and dislocation expression may be harmful to the kidney.

Stunted growth played a role. Clinically, there are isolated polycystic kidney dysplasia and obstructive renal dysplasia.

Parallel cases. Both congenital and experimental single gene mutations can lead to cystic renal dysplasia. These genes

Mutations can change interconnections. Theoretically, mutation can affect: ① embryoid proliferation and differentiation of ureteral catheter.

Expression of peptides and matrix proteins needed for branching; (2) The responsiveness of ureteral catheter to fetal basal signals of posterior kidney; ③ Lose

The expression of urinary catheter can initiate and maintain the protein needed to induce the basal epithelium of renal embryos; ④ Fetal base of posterior kidney is sensitive to these letters.

Reaction ability; ⑤ The ability of the basal cells of ureter bud and posterior kidney embryo to respond to signals [10].

Recently, phosphoinositide glycoprotein gene, abbreviated as GPC3 gene, has been isolated. GPC3 deletion and abundance

Cystic renal dysplasia [1 1]. Although single gene and polygene defects will eventually lead to renal dysplasia, but

Its phenotype may be determined by initial gene regulation disorder or expression change, such as congenital obstructive and cystic.

Renal dysplasia [12, 13]. Polycystic dysplasia kidney with growth factor gene in cystic epithelium and stroma.

Change. Over-expression of angiotensin and transfer growth factor in obstructive developing kidney of mice [14]. study

It has been proved that Pax2 and Bcl-2 are the same factors that promote tubular epithelial degeneration in the region of abnormal renal development.

Overexpression [15, 16]. This study may provide important clues for the pathogenesis of various forms of renal dysplasia.

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